Term
| 4 ways we can examine blood |
|
Definition
peripherial blood film/smear
bone marrow aspirate
bone marrow biopsy
automated: CBC, RBC, platelets |
|
|
Term
| peripherial blood film: method and 3 things it evaluates |
|
Definition
direct exam of blood smear or red cell induces
examines size, shape, color |
|
|
Term
| automatic Hb evaluation: what does it measure, how, two classifications |
|
Definition
concentration of Hb
measured sphectrophotometrically
hypochromatic: low Hb decreases color
normochromatic: normal Hb and color |
|
|
Term
| automatic HCT evaluation: what does it measure, how can it be calculated |
|
Definition
fraction of whole blood that is red cells
MCVxRBC |
|
|
Term
| automatic RBC count: what does it measure, what are the units, what is a normal count, what is a count in anemia |
|
Definition
particle count of TBC
absolute count: 24000-84000 ul
marrow response to anemia is 3% or 15000ul (%RBC must be corrected for degree of anemia (0.5-1.5%) |
|
|
Term
| how is manual RBC count recorded |
|
Definition
|
|
Term
| what are the 6 RBC shapes |
|
Definition
normal
spherocyte
eliiptocytes
target cells
pikilocytosis
schistocytes |
|
|
Term
| what is MCV, what does the number indicate |
|
Definition
average volume per red cell
microcytic: <80
normocytic: 80-100
macrocytic: >100 |
|
|
Term
| what is MCH, how can it be calcuated |
|
Definition
average mass Hb per RBC
HB/RBC |
|
|
Term
| what is MCHC, how can it be calculated, what are the units |
|
Definition
average Hb concentration in red cells
(Hb/HCT)
g/dL |
|
|
Term
| what is RDW, what does the value mean, how is it calculated |
|
Definition
ratio of volume of RBC
large number when cells are different volumes
smaller when their the same size
(SD/MCV) |
|
|
Term
| what is the differential WBC count |
|
Definition
| percent total WBC of a certian type |
|
|
Term
| what is the absolute WBC count |
|
Definition
| lymphocytes, monocytes, PMN, eosinophils, basophils |
|
|
Term
|
Definition
|
|
Term
|
Definition
| platelet distribution width (simillar to RDW) |
|
|
Term
|
Definition
electrophoresis: quantifies
HPLC
isoelectric focusing |
|
|
Term
| explain what Hb is made of |
|
Definition
Hb = globin tetramete + heme
heme = prophyrin ring + Fe atom |
|
|
Term
| what are the 3 types Hb and their prevelance |
|
Definition
HbA (a2,B2) 97%
AbF (a2,Y2) 3%
HbA2 (a2,g2) 1.5% |
|
|
Term
|
Definition
| decreased circulating RBC MASS (mass is hard to measure so we say decreased RBC NUMBER) leads to reduced O2 carrying capacity of the blood |
|
|
Term
| how is anemia evaluated, what are the lab limits for definition in males and females |
|
Definition
evaluate Hb, HCT, RBC count
Hb <13.5 male
Hb <12.5 female |
|
|
Term
| what are the 3 common presenting symptoms in anemia |
|
Definition
| pallor, fatigue, lassitude |
|
|
Term
| why would someone have a severe anemia with little symptoms |
|
Definition
| slow onset, more time to adapt |
|
|
Term
| what are symptoms of anemia due to premature destruction (hemolytic) 3, why are these the characterizing symptoms |
|
Definition
due to increased fee Hb
hyperbilirubinemia
jaundice
pigment gallstnes |
|
|
Term
| what are 2 symptoms of anemia due to ineffective erythropoiesis |
|
Definition
increased Fe absorption
Fe overload |
|
|
Term
| what are 3 symptoms of anemia due to congenital abnormalities |
|
Definition
growth retardation
skeletal abnormalities
cachexia |
|
|
Term
| what are the 4 microcytic anemias |
|
Definition
Fe deficiency anemia
anemia of chronic disease
sideroblastic anemia
thallasemia |
|
|
Term
| what are the 3 macrocytic anemias |
|
Definition
folate deficiency
B12 (cobalamin) deficiency
alcoholism/liver disease anemia |
|
|
Term
| what are the 5 classifications of normocytic anemia |
|
Definition
extrinsic anemia (extracorpuscular/peripherial destruction): extravascular and intravascular hemolysis
intrinsic hemolytic anemia (intracorpuscular/under production)
immune mediated hemolytic anemia
angiopathic hemolytic anemia |
|
|
Term
| what are the 3 normocytic anemia with extravascular hemolysis predominance |
|
Definition
hereditary spherocytosis
sickle cell anemia
hemoglobin C disease |
|
|
Term
| what are the 2 normocytic anemia with intravascular hemolysis predominance |
|
Definition
paroxysmal noctournal hemoglobinuria
G6PDH deficiency |
|
|
Term
| what are the 3 normocytic immune hemolytic anemias |
|
Definition
IgG intravascular hemolysis
IgM extravascular hemolysis
drug induced |
|
|
Term
| what causes RBC to be microcytic |
|
Definition
with each division erythroblasts get smaller and make RBC
in microcytic anemia they go through extra division because there is a DECREASE IN HB PRODUCTION they try and make normal [Hb] by making cells smaller |
|
|
Term
| what are things that cause a decreased Hb production 4 |
|
Definition
Fe deficiency
Fe stuck in macrophages - anemia of chronic disease
decreased protophyrin - sideroblastic anemia
decreased globin - thallassemia |
|
|
Term
| what 12 things cause Fe deficiency anemia |
|
Definition
malnutrition
acute blood loss
chronic blood loss: ulcers, colon cancer, hemorrhoids, hook worm
malabsorption: celiac disease, spruce, gastroectomy
breast feeding
menorrhagia
pregnancy |
|
|
Term
| how does malnutrition cause Fe deficiency anemia (how is it absorbed, transferred, stored) |
|
Definition
consumed as heme (20% absorbed) from meat and veggies (1-2% absorbed)
Fe3+ reduced by ferric reductase to Fe2+
Transported by DMT1 into duodenum enterocytes
Transported on basal side by ferroportin
Regulated by hepcidin (degrades transporter when no Fe is needed)
Some Fe 2+ stays inside the cell when the body dosent need it and is sloughed off with exphoilation of the GI tract
Hephaestin oxidizes Fe2+ to Fe3+
Fe3+ is transported in plasma to store in macrophage on ferritin in liver/bone |
|
|
Term
|
Definition
| prevents fenton Fe reaction from forming free ROS in Fe absorption in duodenum |
|
|
Term
|
Definition
| gate that decides to absorb Fe in duodenum (has to be choosy, we have no Fe elimination process) |
|
|
Term
|
Definition
regulator of ferrportin gate (binds to ferrportin causing degredation)
stops Fe absorption in duodenum |
|
|
Term
|
Definition
|
|
Term
| how long does it take to restore blood volume after acute loss, what anemia will they have until then |
|
Definition
2-3d
normochromic normocytic anemia |
|
|
Term
| what process occurs if someone has had acute blood loss for several days and cannot replenish |
|
Definition
| erythropoetin causes reticulocytosis and more RBC |
|
|
Term
| define chronic blood loss anemia |
|
Definition
| anemia develops when rate of loss > production or Fe stores in body |
|
|
Term
| what disease can caue malabsorption abemia |
|
Definition
|
|
Term
| why does gastrectomy cause anemia |
|
Definition
Fe2+ is easily absorbed and kept charged with acid
less stomach acid means Fe3+ will not be converted and it is less absorbed |
|
|
Term
| what is the most common cause of Fe deficiency in an infant |
|
Definition
| breast milk has little Fe |
|
|
Term
| what is the most common cause of Fe deficiency in a child |
|
Definition
|
|
Term
| what is the most common cause of Fe deficiency in a adult male and female |
|
Definition
peptic ulcers - male
menorrhagia and pregnancy - female |
|
|
Term
| what is the most common cause of Fe deficiency in elderly 2 |
|
Definition
colon polyps - western
hook work - 3rd world |
|
|
Term
| what are the 4 stages of Fe deficiency anemia |
|
Definition
storage Fe depleted, decreased Fe containing proteins
serum Fe depleted
normocytic anemia
microcytic hypochromatic anemia |
|
|
Term
| Fe deficiency anemia: signs of stage 1 Fe storage depletion |
|
Definition
decreased ferritin: less Fe stored, less needed
increased TIBC: macrophages see decreased ferritin and increase transferrin to find Fe |
|
|
Term
| Fe deficiency anemia: signs of stage 2 Fe serum depletion |
|
Definition
decreased serum Fe
decreased percent saturation |
|
|
Term
| Fe deficiency anemia: signs of stage 3 normocytic anemia |
|
Definition
| marrow tries to make more RBC but Fe is low so it makes less, but properlly formed, RBC |
|
|
Term
| Fe deficiency anemia: signs of stage 4 microcytic hypochromatic anemia |
|
Definition
there isnt enough Fe for normal RBC anymore so now RBC have less Hb (and thus less color) and are smaller to keep [Hb] a little higher
increase MCV, MCHC
decreae platelets
increased RDW: non-unison change from normocytic to microcytic widesn range of RBC size
increased free RBC protoporphyrin: it has less Fe to bind to so some is free now |
|
|
Term
| 4 ways to diagnose Fe deficiency anemia |
|
Definition
serum Fe: 3 transferrin hold 1 Fe in blood
total Fe binding capacity (TIBC): measures how much transferrin
percent saturation: how many transferrin-Fe to transferin-X (normally 1/3)
serum ferritin: how much Fe stored |
|
|
Term
| 3 symptoms of mild Fe deficiency anemia |
|
Definition
asymptomatic
weakness
listlessness |
|
|
Term
| plummer vinsin syndrome: cause, 3 signs |
|
Definition
| Fe deficiency anemia with eosphageal web causes dysphagia, atrophic flossitis, beefy red tonge |
|
|
Term
| TX Fe deficiency anemia 2 |
|
Definition
ferrous sulfate
treat cause |
|
|
Term
| 7 causes of anemia of chronic disease |
|
Definition
chronic microbial infection: osteomyelitis, bacterial endocarditis, lung abscess
chronic immune disorders: RA, regional enteritis
neoplasm: hodgkins lymphome, carcinoma of lung and breast |
|
|
Term
| normal Fe vales: intake, loss, total body in men and women |
|
Definition
intake 10-20mg
loss 1-2mg
2g in women
6g in men |
|
|
Term
|
Definition
80% function: 20% catalase, cytochromes. 80% Hb
20% storate as hemoserdin and ferritin in liver, spleen, marrow, muscle |
|
|
Term
| 8 symptoms of severe Fe deficiency anemia |
|
Definition
anemia: pallor
alpplecia
tongue/gastric mucosa atrophy
malabsorption
PICA
kolionychia: spoon shaped nails
hypovolemia: CV collapse, shock, death
plummer vinsin syndrome |
|
|
Term
| pathology of anemia of chronic disease |
|
Definition
chronic inflammation increases HEPCIDIN which sequesters Fe in macrophages and RBC precursors cannot make heme
hepcidin and inflammation also decrease erythropoetin and RBC production
thought is that hepcidin is hiding Fe from being consumed by bacteria |
|
|
Term
| what type of anemia is anemia of chronic disease |
|
Definition
| normocytic normochromatic --> microcytic hypochromatic |
|
|
Term
| 5 lab signs of anemia of chronic disease |
|
Definition
decreased Fe
increased ferritin: inability to store and use Fe
decreased TBIC: transferrin not needed
decreased percent saturation
increased FEP: not enough Fe means increased protophyrin unbound
increased hepcidin: due to pro-inflammagory cytokines |
|
|
Term
| how can anemia of chronic disease and Fe deficiency anemia be distinguished |
|
Definition
Ferritin is low in Fe deficiency and TIBC is high
it is opposite in anemia of chronic disease |
|
|
Term
| explain the normal production of prophyrin |
|
Definition
| succinylCoA + amino lovonic A synthase > aminolobonic acid > + ALAS > prophyrin > + Fe + ferochetylase > heme |
|
|
Term
| cause of hereditary spherocytosis |
|
Definition
defect in ALAS used in heme production
acquired: alchiism (mitochrondia damage), lead poisoning (denatures enzymes), B6 deficiency |
|
|
Term
| pathology of hereditary spherocytosis |
|
Definition
| Fe shows up at mitochondria of monocytes and there is no heme so it gets stuck in ring around mitochondria making RINGED SITEROBLASTS |
|
|
Term
| lab signs of hereditary spherocytosis |
|
Definition
increased ferritin: Fe damages and kills cells releasing Fe into serum whichis eaten by macrophages and attached to ferritin
decreased TBIC
increased serum FE: cells release Fe into serum when they die
increased percent saturation |
|
|
Term
| general cause and purpose of thalassemias |
|
Definition
AR decrease synthesis of globin decreases Hb causing microcytic anemia
derived for protection from PLASMODIUM FALVIPARUM |
|
|
Term
| a-thalassemia: who it effects, what type of Hb, cause |
|
Definition
DELETION of a-globin on CH16
affects ALL Hb types (HbF, HbA, HbA2)
common in southeast asia, african americans |
|
|
Term
|
Definition
silent carrier - 1 deletion
a-thalassemia trait - 2 deletions
HbBart (b4) disease - 3 deletions
HbBart (Y4) disease - 4 deletions |
|
|
Term
| signs of silent carrier a-thalassemia |
|
Definition
| asymptomatic carrier, normal RBC |
|
|
Term
| signs of a-thalassemia trait |
|
Definition
mild anemia
INCREASED RBC count |
|
|
Term
| 2 types of a-thalassemia trait and their population affected, which is worse |
|
Definition
cys (--/aa): asians, high risk of transfer to offspring
trans (a-,a-): more in african americans |
|
|
Term
| 4 lab signs in a-thalassemia trait |
|
Definition
decreased MCV, Hb, HCT
increased RBC count |
|
|
Term
|
Definition
|
|
Term
| signs of HbBart (B4) in utero and after birth, why |
|
Definition
utero: HbF works fine, asymptomatic
birth:
B chains for tetrametes (HgB) causing RBC damage and macrophage consumption
ineffective erythropoesis
severe anemia |
|
|
Term
| how is HbBart (B4) diagnosed |
|
Definition
| HbB seen on electrophoresis |
|
|
Term
| signs of HbBart (Y4), why |
|
Definition
lethal in utero
Y chains form tetrameres (HbB) that damage RBC and have high affinity for O2 but cannot deliver it to tissues |
|
|
Term
| how is HbBart (Y4) diagnosed 3 |
|
Definition
| HbB, HbH, HbPortland on electrophoresis |
|
|
Term
|
Definition
|
|
Term
| B-thalassemia: population, cause, Hb affected |
|
Definition
AA, greeks, italians
MUTATION in B-globin promoter region, coding sequence, splice site (most common) on CH11
affects HbA only |
|
|
Term
|
Definition
(B/B+) B-thalassemia minor (B-thalassemia trait)
(B0/B0) B-thalassemia major (cooley's disease) |
|
|
Term
| signs of B-thalassemia minor |
|
Definition
|
|
Term
| 8 labs supporting B-thalassemia minor |
|
Definition
decreased MCV, HbA, Hb, HCT
increased RBC, HbA2, HbF |
|
|
Term
| pathology of B-thalassemia minor |
|
Definition
target cells have decreased Hb and RBC volume
this allows for excess membrane and blebing
target cells have Hb darkening in central clearing |
|
|
Term
| signsof B-thalassemia major |
|
Definition
6mo after birth (when HbF decreases) severe anemia
HbF often extends beyond normal time |
|
|
Term
| pathology of B-thalassemia major: how does it damage, what are the effects |
|
Definition
a2 chains dimerize with eachother and percipitate (INEFFECTIVE ERYTHROPOESIS: RBC membrane damage and popotosis why bring made)
poorly made cells get EXTRAVASCULAR HEMOLYSIS
massive erytheroid hyperplasia |
|
|
Term
| what is erythroid hyperplsaia |
|
Definition
hematopossis in skull bones causes bone remodeling making crew cut x-ray and chipmunk face
extramedullary hematopoesis and HSM
risk of aplastic crisis with parvo B19 which infects erythroblasts |
|
|
Term
| 9 labs signs supporting B-thalassemia major |
|
Definition
microcytic
hypochromatic
target cells
nucleated RBC
increased RDW, RBC, HbA2, HbF
decreased HbA |
|
|
Term
| treatment of B-thalassemia major |
|
Definition
dependent on chronic transfusion
marrow transplant |
|
|
Term
| SE of tx for B-thalassemia major |
|
Definition
| secondary hematochromatosis (cannot get rid of Fe from transfusions) |
|
|
Term
| what is the cause of macrocytic RBC |
|
Definition
erythroblast does not divide enough due to delay in nuclear DNA maturation, cell division making giant metamyelocytes with NUCLEAR ASYNCHRONY (cytoplasm synthesis > normal rate)
could undergo apoptosis (INEFFECTIVE HEMATOPOESIS) |
|
|
Term
| how is foate acquired and used |
|
Definition
| green veggies > jejunum > tetrahydrofolate methylated > B12 demythelyates > B12 transfers methyl to homocystine > methionine + THF > DNA purine and thymidine production |
|
|
Term
| how does folate deficiency cause anemia, what are some complications |
|
Definition
deficiency causes decreased DNA synthesis and cell division cessation
increased levels of homocystine
body has little stores so anemia develops in months
cause megaloblastic anemia: RBC and other rapidly dividing cells (INTESTINES) become large due to decrease in DNA components
pancytopenia: not isolated anemia, some cells live and some die, but total RBC output decreases |
|
|
Term
| causes of folate deficiency 8 |
|
Definition
poor diet: alcoholic, elderly
increased demand: pregnancy, cancer, hemolysis
folate antagonists: methotrexate
malabsorption: contraceptives, phenytoin |
|
|
Term
| 5 signs of marrow changes in folate deficiency |
|
Definition
thrombocytopenia
megaloblastic: enlarged erythroid precursors
megakaryocytes with large nuclei
metamyelocytes: large granulocyte precursors
granulocytopenia (hypersegmented neutrophils in blood): before anemia develops |
|
|
Term
| 4 signs in blood in folate deficiency |
|
Definition
hypersegmented neutrophils
RBC with egg shaped macro-obalocytes
decreased folate
increased homocystine
normal methylamalonic A and B12 |
|
|
Term
| symptoms of folate deficiency 3 |
|
Definition
flossitis: sore tongue
chlirosis
often complicated with other vitamin deficiencies |
|
|
Term
| explain normal production, absorption, and storage of B12 |
|
Definition
| B12-protein > B12-haptocorrin (from salive) > small intestine > protease cleaves haptocorrin > B12-intrinsic factor (from parietal cells) via cuvulin > ileum > B12-plasma transcovalamin > liver and cells |
|
|
Term
| how does B12 deficiency cause anemia, what are the complications in blood |
|
Definition
onset is long term B12 stores can last 5y
B12 cannot demythelate THF or transfer it to homocystine halting DNA synthesis and causing cell division cesation
causes megaloblastic anemia: RBC and other rapidly dividing cells (INTESTINES) become large due to decrease in DNA components
causes subacute combined degeneration |
|
|
Term
| why does subacute combined degeneration occur with B12 deficiency |
|
Definition
B12 convers methylamolnic A to succinyl CoA
when it dosent methylamonic A builds up on posterior and lateral columns |
|
|
Term
| how can you use degree of subacute combiined degeneration to estimate the anemia levels |
|
Definition
|
|
Term
| cause of B12 deficiency 6 |
|
Definition
pernicious anemia- autoimmune
destruction of parietal cells in stomach
pancreas insufficiency (cannot cleave B12 from carriers)
damage to terminal ileum
dietary deficiency
gastric atrophy in elderly interferes with pepsin production so B12 isnt released |
|
|
Term
| explain why pernicious anemia leads to B12 deficiency |
|
Definition
| autoimmune reaction to parietal cells and intrinsic factor make it so B12 isnt absorbed |
|
|
Term
| what antibodies can cause B12 deficiency 3 |
|
Definition
B12 blocking antibodies: prevent binding fo cubulin
parietal canicular antibodies: attack parietal cells
ileal receptor blocking antibodies |
|
|
Term
| what conditiosn is pernicious anemia associated with 3 |
|
Definition
hashimotos thyroiditis
addisons disease
DM1 |
|
|
Term
| what are some marrow signs that support B12 deficiency 5 |
|
Definition
megaloblastic: enlarged marrow erythroid precursors
megakaryocytes with large nuclei
metamyelocytes: large granulocytic precursors
granulocytopenia: hypersegmented neutrophils in the blood before anemia develops
thrombocytopenia |
|
|
Term
| what are some blood signs that support B12 deficiency 6 |
|
Definition
auto-antibodies in serum and gastric juice
hypersegmented neutrophils
RBC with egg shaped macro-ovalocytes
decreased B12 (folate normal)
increased homocystine
INCREASED METHYLMALONIC A |
|
|
Term
| what are some symptoms of B12 deficiency 6 |
|
Definition
glossitis
chilosis
burning, tingling, numbness in hands and feet
ataxia
decreased position sense (esp toes)
jaundice |
|
|
Term
| treatment of B12 deficiency |
|
Definition
reticulocyte response in 2-3d to parentrail B12
WILL RESPOND TO FOLATE THERAPY BUT THAT WILL ONLY CORRECT THE ANEMIA NOT CORD DEGENERATION |
|
|
Term
| how can alcoholism and liver disease caused anemia be distinguished from B12 or folate deficiency anemia |
|
Definition
| causes megaloblastic anemia too but only RBC become large NOT other rapidly dividing cells like intestines |
|
|
Term
| how is extrinsic hemolytic anemia diagnosed |
|
Definition
in anemia reticulocyes >3% due to increased erythropoetin and reticulocytosis
RBC are large, blue cytoplasm due to residual RNA |
|
|
Term
| causes of extrinsic hemolytic anemia 3 |
|
Definition
immune mediated
mechanical destruction
infection |
|
|
Term
| patholog of extrinsic hemolytic anemia, what changes does it cause in the body, how is it started |
|
Definition
RBC damaged or immunologically rargeted
reticuloendothelial system destories RBC
globin breaks into AA
heme: Fe is recycled and protophorin turned into unconjugated bilirubin which binds albumin and sits in blood (jaundice) until it can be conjugated in liver
liver conjugates and makes bile with too much bilirubin which makes gall stones |
|
|
Term
| 5 signs of extrinsic hemolytic anemia |
|
Definition
reactive hyperplasia; marrow and spleen hyperplasia (HSM)
anemia
jaundice
pigmented gall stones
haptoglobin decreased because some Hb leaks from macrophages onto plasma so lactate DH is increased when it binds |
|
|
Term
| cause or intravascular hemolysis, process of destruction |
|
Definition
RBC lysed in serum due to mechanical damage (valve, complement, toxins, heat)
Hb in serum binds to haptoglobin, floats around in blood
evuntally removed in spleen |
|
|
Term
| signs of intravascular hemolysis |
|
Definition
hemoglobinemia
hemoglobinuria
hemosiderinuria
heptoglobin is used to bind Hb so its decreased and releases lactte DH which increases ins erum
unconjugated hyperbilirubinemia
acute tubular necrosis of kidney: Hb and RBC membrane stroma is toxic
reactive hyperplasia: marrow and spleen hyperplasia (HSM) |
|
|
Term
| howis intrinsic hemolytic anemia diagnosed |
|
Definition
in anemia reticulocytes will not rise >3% because the marrow cannot produce them
NOTE: reticulocytes can seem elevated in anemia falsly, must use correction factor formula
%reticulocytes = HCT/45) |
|
|
Term
| cause of intrinsic hemolytic anemia |
|
Definition
inherited membrane abnormalities
enzyme deficiencies
Hb synthesis dosorders |
|
|
Term
| howis intrinsic hemolytic anemia diagnosed |
|
Definition
in anemia reticulocytes will not rise >3% because the marrow cannot produce them
NOTE: reticulocytes can seem elevated in anemia falsly, must use correction factor formula
%reticulocytes = HCT/45) |
|
|
Term
| cause of intrinsic hemolytic anemia |
|
Definition
inherited membrane abnormalities
enzyme deficiencies
Hb synthesis dosorders |
|
|
Term
| cause of hereditary spherocytosis, pathology, how it causes problems |
|
Definition
AD (most common) AR more severe
deformed spectrin, ankyrin, or band 3/4.2 in cytoskeleton of RBC weakens vertical interaction between membrane and cytoskeleton and causes blebs which get eaten in spleen and deform so the cell becomes SPHEROCYTE
evuntally their so small they cannot manuever in spleen so it consumes them via extravascular hematopoesis
some intravascular hematopoesis occurs due to abnormal RBC shape
this happens at all different stages of RBC so their is a wide range in RBC size |
|
|
Term
| 3 lab signs of hereditary spherocytsis |
|
Definition
spherocytes: round cells with loss of central palloe
increased RDW
increased MCHC: cells decrease in size but still have same Hb content increaseg [Hb] |
|
|
Term
| 6 symptoms of hereditary spherocytosis and why |
|
Definition
anemia: 30% asymptomatic
splenomeagly: work hypertrophy (500-1000g) and congestion of splenic cords with macrophages
hemolytic crisis: spleen destruction due to intercurrent event (like mono)
jaundice: increased RBC destruction causes more bilirubin conjugation in liver and a back up of unconjugaed bilirubin in blood
pigment gall stones: (chorlelithiasis) increase in bilirubin processing puts more into bile causing stones
aplastic crisis: parvo B19 invades erythroid precursors worsening anemia
hemosiderosis: excess heme from RBC destruction is taken up into tissues
erythroid hyperplasia of marrow and reticulocytes |
|
|
Term
| diagnosis of hereditary spherocytosis |
|
Definition
osmotic fragility test
RBC + hypotonic solution causes spherocyte (normal)
spherocyte + hypotonic solution causes lysis |
|
|
Term
| treatment of hereditary spherocytosis, prognosis and complications |
|
Definition
splenectomy: spherocytes persist but are not depleted
see howell jolly bodies (spleen isnt there to remove DNA gragments from new RBC so the persist) |
|
|
Term
| cause of sickle cell anemia |
|
Definition
AR mutation in B0Hb (glutamic acid - hydrophillic --> valine - hydrophobic)
derived for protection from FALCIPARUM MALARIA |
|
|
Term
| what are the 2 types of sickle cell |
|
Definition
1 gene mutation - heterozogoys
2 gene mutation - homozygous |
|
|
Term
| what are the RBC changes in heterozygous sickle cell, the symptoms, and why |
|
Definition
55% Hb and 45% HbS: you need 50% HbS to cause sickle except in renal medulla where it is low O2 and hypertonic which allows sickle and causes microinfarcts
microscopic hematuria and decreased ability to concentrate urine |
|
|
Term
| signs of homozogous sickle cell 11 |
|
Definition
sicking of cells
extravascular hemolysis/target cells
extramedullary hematopoesis/massive erythroid hypoplasia
aplastic crisis
intravascular hemolysis
microvascular vasooclusion
stroke
seizure
aseptic necrosis of femur head
leg ulcers
Ca bilirubin gall stones |
|
|
Term
| give two reasons sickle cell could be benificial to the patient |
|
Definition
protection from falciprum malaria
coexistance with a-thalassemia decreaes Hb and reduces sickling |
|
|
Term
| give 7 reasons that cause sickling and why |
|
Definition
acidosis: inflammation and infection
inflammation: causes cells to be more sticky
low O2: sluggish capillary flow and sickling esp in spleen and marrow
increases in HBS: more stuck together. sickling causes influx of Ca and efflux of K and water. DEHYDRATION damages membrane skeleton and causes sticking and sickling
HbS interacts with HbA weakly
HbF INHIBITS HbS polymerization - no sickling
HbC aggregates with HbS
increased MCHC (Hb) causes more sickling (decreased MCHC like in a-thalassemia causes less) |
|
|
Term
| why do sickle cells go through extravascular hemolysis, explain the process |
|
Definition
they continously sickle and desicle as they go through normal oxygenation/deoxygenation which slowly damages the cells
the spleen removes them or they lysis releasing haptoglobin and making TARGET CELLS (membrane damages causes dehydration and the membrane blebs creating bleb in center with Hb in it) |
|
|
Term
| why is there massitve erythroid hypoplasia and extramedullary hematopoesis in sickle cell, what are the signs of this |
|
Definition
RBC are destoried and non-functional so their is less effective RBC in circulation so bone tries to make more but the normal long bones can't keep up so other bones help out
this causes chipmunk facies and cerw cut xray |
|
|
Term
| why do people with sickle cell get aplastic crisis |
|
Definition
| when infected with parvo B19 it infects the little RBC precursors they have decreasing oxygenation even more |
|
|
Term
| why do people with sickle cell get intravascular hemolysis |
|
Definition
| minor, due to mechanical fragility of sickled cells |
|
|
Term
| what are 5 effects of vasoocclusion in sickle cell |
|
Definition
infarct and fibrosis of spleen
dictylitis
vasoocclusion of lung
renal papillary necrosis
pain crisis |
|
|
Term
| what are the complications of vasooclusion in the spleen in sickle cell 4 |
|
Definition
decreased antibody formation allows infection of encapsulated microbes (MOST COMMON COD IN KIDS)
autosplenectomy: fibrosis of spleen causes regression (CHRONIC ERYTHROSTASIS) leading to formation of HOWEL JOLLY BODIES in RBC (spleen no longer removes residual RNA from new RBC)
sequestration crisus: rapid enlargement due to entraped RBC causing HYPOVOLEMIC SHOCK |
|
|
Term
| what microbes can infect when the spleen stops working in sickle cell, what is a common infection complication |
|
Definition
encapsulated microbes: pneumococcus, H. influenza)
salmonella osteomyelitis |
|
|
Term
| what is the most common COD in kids with sickle cell |
|
Definition
| spleen damage due to vasoocclusion causes decreased antibody formation and allows infection with encapsulated microbes |
|
|
Term
|
Definition
| swelling and infarct of bones in hands and feet (common in 6-9mo sickle cell patients) |
|
|
Term
| what are the complications of vasoocclusion in the lung in sickle cell 4 |
|
Definition
acute chest syndrome
CP
SOB
infiltrates
MOST COMMON COD IN ADULTS |
|
|
Term
| what is the cause and signs of acute chest syndrome |
|
Definition
| pneumonia causes vasodilation which increases RBC transit and dehydration, acidemia, and deoxygenation of cells increasing sickling |
|
|
Term
| what are the complications of renal papillary necrosis in sickle cell 2 |
|
Definition
hematuria
loss of concentrating ability |
|
|
Term
|
Definition
| hypoxic tissue injury and infarct due to clogging of sickle cells in small vessels causes severe pain |
|
|
Term
| what is the most common COD in adults with sickle cell |
|
Definition
|
|
Term
| treatment for sickle cell, MOA |
|
Definition
hydroxyriua after 6mo
gentile inhibitor of DNA synthesis increases HBF and decreases leukocytes which decreases inflammation
increases MCV and MCHC due to decreased DNA synthesis
causes NO production leading to vasodilation and inhibition of clotting |
|
|
Term
| 3 types of screening for sickle cell |
|
Definition
prenatal screen: fetal DNA detection of point mutations
metabolisulfite screen: causes sticking of ANY HbS (even heterozygous)
Hb electrophoresis: thicker band means more of that type of Hb |
|
|
Term
| what is the distribution of Hb in homozygous sickle cell disease |
|
Definition
90% HbS
8% HbF
2% HbA2
0% HbA |
|
|
Term
| what is the distrubution of Hb in heterozygous sickle cell trait |
|
Definition
|
|
Term
| cause of hemoglobin C disease |
|
Definition
| AR mutation in Hb B-chain (glutamic acid --> lysing) making HbC |
|
|
Term
| how does HbC cause anemia |
|
Definition
| HbC has a greater tendancy to aggregate with HbS so even heterozygous sickle cell disease will have sicking |
|
|
Term
|
Definition
| mild anemia due to extravascular hemolysis |
|
|
Term
|
Definition
|
|
Term
| paroxysmal nocturnal hemaglobinuria: cause |
|
Definition
normally RBC protect themselves from complement damage with DAFT and MIRL which is held on by PIG/GIP
ACQUIRED deficit in myeloid stem cell so platelets, RBC, and WBC have no PIG/GPI which allows for complement destruction of RBC |
|
|
Term
| why is paroxysmal nocturnal hemaglobinuria called that |
|
Definition
when we sleep we breathe shallow and retain CO2 causing resiratory acidosis which activates complement increasing RBC lysis
leads to dark urine in morning due to RBC breakdown products |
|
|
Term
| 8 signs of paroxysmal nocturnal hemaglobinuria |
|
Definition
paroxysmal nocturnal hemaglobinuria
hemoglobinemia
hemoglobuinuria (episodic)
hemosiderinuria (days after episode)
VENOTHROMBOSIS
Fe deficiency anemia
APLASTIC ANEMIA in 10%
decreased PMN allowing for infection |
|
|
Term
| why does paroxysmal nocturnal hemaglobinuria get venothrombosis |
|
Definition
| pt is in procoagulable state due to activity of MAC attack |
|
|
Term
| why does paroxysmal nocturnal hemaglobinuria pt get Fe deficiency anemia |
|
Definition
| Fe is lost in urine with Hb |
|
|
Term
| 4 diagnostic tests for paroxysmal nocturnal hemaglobinuria |
|
Definition
sucrose hemolysis test (sugar water test) - screening: sugar enhances complement attachment
acidified serum test (ham test) - conformation: activates alternate complement pathway
peripherial blood: normocytic anemia with pancytopenia (microcytic Fe deficiency develops later)
decreased CD55 (DAFT) on RBC surface |
|
|
Term
| TX paroxysmal nocturnal hemaglobinuria |
|
Definition
| targeted against MAC attack decreasing hemolysis and thrombotic complications but increasing risk of Neisseria infection (esp menngococcal sepsis) |
|
|
Term
| cause of G6PDH deficiency anemia |
|
Definition
RBC in liver undergo much oxidative stress and must protect themselves with glutathione
GSH + peroxide + glutathione > GS-SG + NADPH > GSH > repeat
NADPH comes from glucose-6-P A varient DH reaction
in G6PDH deficiency there is X-linked mutation in G6PDH A varient
derived for protection from FALCIPRUM MALARIA |
|
|
Term
| what are the two types of G6PDH anemia and who gets them, what are their severities, why |
|
Definition
african (A-) variant (in 10% of AA): mildly decreased t1/2 of G6PDH (only older RBC destoried. less severe
mediterranean varient: very short t1/2 G6PDH (any RBC >30d old is destoried, worse disease) |
|
|
Term
| why does G6PDH deficiency cause anemia |
|
Definition
cells destoried during oxidative stress when ROS increases
destruction causes Hb to percipitate leaving HEINZ BODIES in the RBC which partially destoried in spleen making BITE CELLS
bite cell irregular shape makes them sensitive to intravascular hemolysis |
|
|
Term
| what types of oxidative stress can cause a problem in G6PDH deficiency anemia 4 |
|
Definition
infection
drugs: primaquine, chloroquine, sulfonamide
fava beans |
|
|
Term
| signs of G6PDH deficiency anemia 3 |
|
Definition
occur 2-3d after oxidative stress
hemoglobinuria
back pain |
|
|
Term
| why do people with G6PDH anemia get back pain |
|
Definition
| nephrotoxic Hb in kidney 2-3d after oxidative stress |
|
|
Term
| how is G6PDH deficiency anemia diagnosed 2 |
|
Definition
heinz bodies: shows percipitated Hb
enzymatic studies: must do AFTER acute episode or all the cells left over will just be good cells and give no results |
|
|
Term
| causes of warm imune hemolytic anemia 4 |
|
Definition
primary: idiopathic
secondary: leukemia, lymphoma, neoplasm autoimmune (esp SLE) |
|
|
Term
| how does warm immune hemolytic anemia cause anemia |
|
Definition
IgG binds RBC in WARM areas of the body causing complement C3b recruitment
membrane consumption in spleen bit by bit over time makes cells SPHEROCYTE shape
most hemolysis occurs due to opzonixation of RBC by autoantibodies INTRAVASCULAR HEMOLYSIS |
|
|
Term
| signs of warm immune hemolytic anemia |
|
Definition
jaundice
spenomeagly
normocytic anemia with spherocytes
coombs test |
|
|
Term
| explain how a direct and indirect coombs test for wam immune hemolytic anemia works |
|
Definition
direct: checks RBC: antibody against IgG causes agglutination
indirect: checks serum: put RBC in pt serum and add anti-IgG causing agglutination |
|
|
Term
| tx warm immune hemolytic anemia |
|
Definition
steroids
IVIG
remove drug
splenectomy |
|
|
Term
| cause of cold immune hemolytic anemia 4 |
|
Definition
mycoplasma pneumonia
infectious mono
idiopathic
lymphoid neoplasms |
|
|
Term
| how does cold immune hemolytic anemia cause anemia |
|
Definition
in cold (ears, hands, toes) IgM binds RBC and fixes C3b but isn't lysed because its too cold for the rest of the complement cascade
when they get to warmer place IgM is released by C3b stays.
this causes cell to be eaten by macrophages, EXTRAVASCULAR HEMOLYSIS, and causes agglutination with other cells |
|
|
Term
| signs of cold immune hemolytic anemia 7 |
|
Definition
hepatosplenomeagly
raynauds phenomenon
positive DAT for C4b
intravascular hemolysis: hemoglobinuria, hemoglobinemia, decreased haptoglobin
positive coombs test |
|
|
Term
| how can penicillin cause immune hemolytic anemia |
|
Definition
| absorbs into RBC and antibody binds cell leading to extravascular hemolysis |
|
|
Term
| how can quinidine cause immune hemolytic anemia |
|
Definition
| makes drug-Ab complexes that are absorbed into RBC causing complement fixation and intravascular/extravascular hemolysis |
|
|
Term
| how can a-methyldopa cause immune hemolytic anemia |
|
Definition
| induces RBC autoantibody (same as warm/IgG but self limiting in a few mo) |
|
|
Term
| how can cephalosporin cause immune hemolytic anemia |
|
Definition
| causes RBC membrane to become stickly and IgG and complement adhere non-specificially |
|
|
Term
| what are the two types of angiopathic hemolytic anemia |
|
Definition
macroangiopathic hemolytic anemia (in large vessels)
microangiopathic hemolytic anemia (in capillaries) |
|
|
Term
| causes of macroangiopathic hemolytic anemia 2 |
|
Definition
calcified stenotic aortic valve: sheer forces due to turbulent flow and abnormal pressure gradients
artificial mechanical heart valves |
|
|
Term
| causes of microangiopathic hemolytic anemia 6 |
|
Definition
DIC - most common
thrombotic thrombocytopenic purpura
hemolytic uremic syndrome
malignant HTN
SLE
disseminated cancer |
|
|
Term
| how does how is angiopathic hemolytic anemia diagnosed |
|
Definition
| small vessel thrombosis partially blocks vessels and sheers RBC causing SCHISTOCYTES (HELMET/TRIANGLE/BURR RBC) |
|
|
Term
| what are the causes of anemia due to underproduction 6 |
|
Definition
microcytic and macrocytic anemia causes decreased reticulocytes
renal failure decreases erythropoetin and thus reticulocytes
damaged progenitor cells
drugs and chemicals
viruses
autoimmune / idiopathic 50% |
|
|
Term
| what are the causes of damaged RBC progenitor cells 2 |
|
Definition
parvovirus B19
aplastic anemia |
|
|
Term
| how does parvovirus B19 effect RBC |
|
Definition
stops erythropoesis and causes anemia in pt with preexisting marrow stress
self limited |
|
|
Term
| drugs that cause aplastic anemia |
|
Definition
antioneoplastic
benzene
chlorphenicol reaction or hypersensitivity |
|
|
Term
| viruses that cause aplastic anemia, how can you tell cause is viral |
|
Definition
community acquired viral hepatitis (not ABC)
aplasia for several months after |
|
|
Term
| 2 autoimmune causes of aplastic anemia |
|
Definition
familial telomere defect causes premature senescence of hematopoetic stem cells
autoreactive T cells: associated with paroxysmal nocturnal hemoglobinuria, T cells may target PIG/GIP |
|
|
Term
| blood changes in aplastic anemia 7 |
|
Definition
marrow failure/hepatocellular/fatty marrow: 10% cellularity only WBC and plasm a(dry tap)
pancytopenia: normocytic normochromatic anemia with decreased reticulocyte count
anemia
thrombocytopenia
granulocytopenia
hemorrhage
bacterial infection |
|
|
Term
| symptoms of aplastic anemia 4 |
|
Definition
petechiae and echimosis
palor, weakness, listless
chills and fever
infections |
|
|
Term
|
Definition
stop drug
transfusion
marrow stimulation
immune supression
transplant is curative |
|
|
Term
| cause of myelophtisic process |
|
Definition
| pathological process replaces marrow causing decreased hematopoesis and pancytopenia |
|
|
Term
| what conditiosn is myelophistic process associated with 7 |
|
Definition
breast, lung, prostate, and other cancer
TB
lipid storage diseases
osteosclerosis |
|
|
Term
| changes in blood in myelophistic process 3 |
|
Definition
misshaped RBC resemble teardrops
immature granulocytic and erythrocytic precursors (leukoerythroblastosis)
thrombocytopenia |
|
|
Term
|
Definition
| increase in blood concentration of RBC usually correlated with increased HB concentration |
|
|
Term
| definition and causes of reactive polycythemia |
|
Definition
| hemoconcentration due to decreased plasma volume caused by dehydration |
|
|
Term
| define absolute polychthemia |
|
Definition
| increase in total RBC mass |
|
|
Term
| 4 causes of primary absolute polycyhemia |
|
Definition
autonomous proliferation of myeloid stem cells
abnormal proliferation of myeloid stem cells
normal/low erythropoetin levels (polychthemia vera)
inherited activating mutations of erythropoetin receptor (rare_ |
|
|
Term
| 3 appropirate causes of secondary absolute polychthemia |
|
Definition
| lung disease, high altitude, cyanotic heart disease |
|
|
Term
| 4 inappropirate causes of secondary absolute polychthemia |
|
Definition
erythropoetin secretin tumor
carcinoma hepatoma
cerebellar hemagiioblastoma
renal cell carcinoma |
|
|
Term
| in general, what is the cause of secondary absolute polycythemia |
|
Definition
| response to increased erythropoetin |
|
|
Term
| what is the normal, risk of post-traumatic bleeding, and risk of spontaneous bleeding number of platelets |
|
Definition
150000-450000 platelet/ul is normal
20000-50000 increased risk of post trauma bleeding
<20000 risk of spontaenous bleeding |
|
|
Term
| what does prothrombin time assess 7 |
|
Definition
extrinsic and common pathways
factors VII, V, II, I
warfarin therapy |
|
|
Term
| what does partial thromboplastin time assess 11 |
|
Definition
intrinsic and common pathways
factors XII, XI, IX, VIII, X, V, II, I
heparin therapy |
|
|
Term
| what does bleeding time measure |
|
Definition
|
|
Term
| what does fibrin split products measure |
|
Definition
| cleavage of fibrin or fibrinogen |
|
|
Term
|
Definition
| clevage of fibrin (htey hold fibrin together) |
|
|
Term
| what do factor levels measure |
|
Definition
| individual factor activity |
|
|
Term
| where and from what are platelets made |
|
Definition
megakaryocytes
in the marroe |
|
|
Term
| what are 3 ways platelet production can be stopped in the marrow |
|
Definition
marrow disease
impaired platelet production
ineffective megakaryopoiesis |
|
|
Term
| what are two marrow diseases that stop platelet production (give some examples of each) |
|
Definition
aplastic anemia: congenital, acquired
marrow infiltration: leukemia, disseminated cancer |
|
|
Term
| what are two ways to impair platelet production in the marrow, give some examples of each |
|
Definition
drugs: alcohol, thiazides, cytotoxic
infection: measles, HIV |
|
|
Term
| what is the most common hematological manifestation of HIV |
|
Definition
| impaired platelet production |
|
|
Term
| what are two things that cause ineffective megakaryopoiesis |
|
Definition
megaloblastic anemia
paroxysmal nocturnal hemoglobinuria |
|
|
Term
| explain the process of the formation of a primary platelet plug |
|
Definition
vessel damage
vasoconstriction: neural stimulation tells endothelial cells to release endothelian
vWF: released from subendothelial collagen webiel palate bodies when exposed during damage
platelets bind vWP with GP1b receptor (adhesion) then degranulate "releasing action" |
|
|
Term
| what are the 6 things platelets release upon activation via vWF |
|
Definition
ADP
fibrinogen
phospholipase A2
serotonin
platelet factor 4
platelet derived GF |
|
|
Term
|
Definition
| causes platelets to express GP IIb/IIIa allowing for aggregation |
|
|
Term
|
Definition
| links platelets do eachother |
|
|
Term
| what does phospholipase A2 released from platelets do |
|
Definition
combines with cyclooxygenase, thromboxane synthase, and arachadonic acid making...
cyclic endoperoxidase and THROMBOXANE A2 which recruits more platelets |
|
|
Term
| what does serotonin released from platelets do |
|
Definition
| causes vasospasm in coronary vessels |
|
|
Term
| what does platelet factor 4 do |
|
Definition
| glycoprotein that neutralizes anticoagulation of heparin |
|
|
Term
| what does platelet derived GF do |
|
Definition
| mitogen that initiates smooth muscle cell proliferation causing athlerogenesis |
|
|
Term
| why do we need secondary hemostasis if we have a platelet plug |
|
Definition
| platelet plugs are too weak |
|
|
Term
| what are the two categories of disorders of primary hemostasis |
|
Definition
| quantative and qualitative |
|
|
Term
| what are the signs of a quantative platlet disorder 13 |
|
Definition
skin bleeding: petechiae, purpura, ecchymosis
bleeding: easy bruising, epistaxia, hemoptysis, GI bleeds, hematuria, menorrhagia
intracranial bleeding: when severe
normal PT/PTT
thrombocytopenia via consumptive coagulopathy
prolonged bleeding time |
|
|
Term
| what are signs of a qualitative platelet disorder 7 |
|
Definition
bleeding: easy bruising, nose bleeds, excess bleeding from trauma, menorrhagia
PT/PTT normal
bleeding time prolonged
thrombocytopenia |
|
|
Term
| what are 6 diseases or conditions that cause quantitative primary hemostasis disorders |
|
Definition
- increased vessel fragility
- systemic conditions activate or damage endothelial cells
- immune thrombocytopenia purpura (ITP)
- thrombotic microangiopathies
- immune destruction
- non-immunologic other |
|
|
Term
| what are the 2 throbotic microangiopathies |
|
Definition
thrombotic thrombocytopenia purpura (TPP)
hemolytic uremic syndrome (HUS) |
|
|
Term
| what are 2 causes of qualitative platelet disorders |
|
Definition
|
|
Term
| 11 causes of increased vessel fragility |
|
Definition
scurvy
amyloidosis
chronic glucicorticoids
inherited CT disorders
infection
hypersensitivity vasculitides
meningococcemia
infective endocarditis
rickettsia
typhoid
henoch-schonlein purpura |
|
|
Term
| 3 systemic conditions that activate or damage endothelial cells |
|
Definition
paraxodical consumptive coagulopathy
severe bleeding |
|
|
Term
| cause of immune thrombocytopenic purpura in adults |
|
Definition
autoimmune platelet IgG to GP IIb/IIIa or GPI made in spleen
bound platelets are consumed by macrophages in the spleen causing thrombocytopenia
usually due to SLE |
|
|
Term
| immune thrombocytopenic purpura cause in kids |
|
Definition
occurs weeks after a viral infection
resolves in a few weeks |
|
|
Term
| immune thrombocytopenic purpura signs 3 |
|
Definition
pregnancy: short lived thrombocytopenia in offspring
intrecerebral and subarachnoid hemorrhages
increased megakaryocytes on marrow biopsy |
|
|
Term
| tx immune thrombocytopenic purpura 3 |
|
Definition
steroids: kids respond well, adults often relapse
IViG: short lived
splenectomy: eliminate source of AB and site of destruction of platelets |
|
|
Term
| general signs of a thrombocytic microangiopathy 4 |
|
Definition
microangiopathic hemolytic anemia
fever
anemia with schistocytes
increased megakatyocytes |
|
|
Term
| cause of thrombotic thrombocytopanie purpura |
|
Definition
autoantibody to metalloprotease ADAMSTS12 stops breaking of vWF into monomeres causing abnormal platelet adhesion
when RBC pass they shear making schistocyte causing microangiopathic hemolytic anemia
damage from RBC causes consumptive coagulopathy and thrombocytopenia |
|
|
Term
| 3 signs specific to thrombotic thrombocytopenia purpura |
|
Definition
CNS abnormalities
renal failure
fever |
|
|
Term
| TX thrombotic thrombocytopena purpura |
|
Definition
plasma exchange/plasmaphoresis (removes autoantibody(
80% successful |
|
|
Term
| cause of hemolytic uremic syndrome |
|
Definition
consumption of E. coli 0517h7 that releases shiga like toxin damages endothelial cells in kidney and brain allowing for microthrombi
this leads to RBC lysis on thrombi causing schistocytes and microangiopathic hemolytic anemia
damage from shiga like toxin and sheared RBC causes endothelial damage and consumptive coagulopathy and thrombocytopenia |
|
|
Term
| 2 signs specific to hemolytic uremic syndrome |
|
Definition
dysentery: mucous diarrhea
renal insufficiency |
|
|
Term
| prognosis of hemolytic uremic syndrome |
|
Definition
| all is reversible except for renal damage |
|
|
Term
| what are causes for immune destruction of platelets 5 |
|
Definition
isoimmune: post transfusion, neonatal
infection: mono, HIV, CMV |
|
|
Term
| what are causes for non-immunologic "other" destruction or removal of platelets 3 |
|
Definition
giant hemangioma
sequestration: hypersplenism
dilutional: HIV |
|
|
Term
| how does aspirin decrease quality of platelets function |
|
Definition
| irreversibly inactivate cyclooxygenase decreasing thromboxane A2 production and thus decreasing aggregation |
|
|
Term
| how does uremia decrease quality of platelet function |
|
Definition
| poor kidney function causes N to build up in body and cause adhesion and aggregation disorders |
|
|
Term
| where are clotting factors made |
|
Definition
| made in liver and released inactive into the blood |
|
|
Term
| how are clotting factors activated 3 |
|
Definition
activating substance
phospholipid surface (platelets)
calcium |
|
|
Term
| what is the function of the extrinsic pathway of coagulation |
|
Definition
|
|
Term
| what is the function of the intrinsic pathway of coagulation |
|
Definition
| fibrin growth and maintenance |
|
|
Term
| how is the intrinsic pathway of coagulation initiated |
|
Definition
| factor XII (hageman) comes in contact with collagen and is converted to XIIA |
|
|
Term
| how is the extrinsic pathway of coagulation initiated |
|
Definition
| tissue factor (III) released via tissue injury comes in contact with factor VII (stable) and with the help of Ca and K is turned into VIIA |
|
|
Term
| what factors and cofactors have a role in the intrinsic pathway of coagulation 12 |
|
Definition
XII
XI
IX
X
II
I
IV (calcium)
VII
K (potassium)
XIII (fibrin stabilizing factor)
V (labile)
PF3 |
|
|
Term
| what factors and cofactors have a role in the extrinsic pathway of coagulation |
|
Definition
III (tissue factor)
VII (stable)
X
II
I
XII (fibrin stabilizing)
PF3
V (labile)
IV (calcium)
K (potassium) |
|
|
Term
| explain the coagulation cascade once it converges to the end |
|
Definition
Xa converts prothrombin (II) to thrombin (IIA) using V, Ca, PF3, and K
thrombin (IIA) converts fibrinogen (I) into fibrin (IA) using XIII and Ca |
|
|
Term
| explain the extrinsic pathway up until the conversion |
|
Definition
VII (Stable) is converted to VIIA using tissue factor (III), Ca, and K
VIIA converts X to Xa using Ca |
|
|
Term
| explain the intrinsic pathway up until the conversion |
|
Definition
collagen turns XII into XIIA
XIIA turns XI into XIA
XIA turns IX (christmas) into IXA using VII, Ca, K
IXA turns X into XA using Ca and K |
|
|
Term
| what are the clinical signs of a disease of secondary hemostasis 7 |
|
Definition
deep tissue bleeding into muscles and joints
easy bruising
deep tissue hematoma
lower extremity joint hemorrhages (hemoarthoses)
rebleeding after surgery
PTT/PT prolonged
bleeding time normal |
|
|
Term
| what are 5 diseases of secondary hemostasis |
|
Definition
hemophilia A- most common hereditary disorder WITH SERIOUS BLEEDING
hemophelia B / christmas disease
coagulation factor inhibitor
vitamin K deficiency
parenchymal liver disease |
|
|
Term
| cause of hemophelia a and how is severity determined |
|
Definition
X-linked or 30% sporatic mutation in factor VIII
severe <1% VIII
moderate 1-5% VIII
mold >5% VIII |
|
|
Term
|
Definition
easy bruising
massive hemorrhages after trauma or operation
hemarthroses: joint bleeds that progress to deformities
NO petechiae |
|
|
Term
| hemophelia A: PTT, PT, platelet count, bleeding time |
|
Definition
increased PTT (improves with normal serum)
normal PT
normal platelet count
normal bleeding time
decreased factor VIII |
|
|
Term
|
Definition
| recombinant factor VIII infusion |
|
|
Term
| prognosis of hemophelia A |
|
Definition
| 15% develop neutralizing antibodies against VIII which is most severe form |
|
|
Term
|
Definition
| x-linked factor IX deficiency resembling hemophelia A |
|
|
Term
| hemophelia B: PTT, bleeding time, PT |
|
Definition
PTT prolonged
PT normal
bleeding time normal |
|
|
Term
|
Definition
|
|
Term
| cause of coagulation factor inhibitor of clotting |
|
Definition
| acquired antibody against coafulation factor impairs function (usually VIII) |
|
|
Term
| coagulation factor inhibitor of clotting: PTT, PT |
|
Definition
PT normal (usually, depends on factor)
PTT abnormal, dosent correct with normal serum addition |
|
|
Term
| how is vitamin K acquired and used for clotting |
|
Definition
generated in gut bacteria
absorbed in liver
activated by epoxide reductase
gamma carboxylates factors II, VII, IX, X |
|
|
Term
| what causes vitamin K deficiency |
|
Definition
abnormalities in bacteria
decreased vitamin K uptake
often in newborns or people on antibiotics |
|
|
Term
| why does parenchymal liver disease affect clotting |
|
Definition
decreased production of coagulation factors
decreased production of epoxide reductase so vitamin K isnt activated |
|
|
Term
| signs of parenchymal liver disease caused bleeding disorder |
|
Definition
|
|
Term
| what are 3 disease that affect primary and secondary hematostasis |
|
Definition
von willebrand disease - most common inherited coagulation disorder
disseminated intravascular coagulation
heparin induced thrombocytopenia (HIT) |
|
|
Term
| in general what is the cause of vWF diseae |
|
Definition
AD genetic vWF deficiency causes problems with platelet adhesion and factor VII stabilization
does not cause clinical signs of secondary hematostasis |
|
|
Term
| what are the 4 types of vWF disease |
|
Definition
|
|
Term
| what are the causes of the 4 types of vWF disease |
|
Definition
1. AD (classic) reduced quantity of vWF and decreased factor VIII levels
2. high moleculear weigh multimeres not made and their most active causing a qualitative vWF deficiency
2b: abnormal HMWM made but removed causing mild thrombocytopenia
3. homozogous inheritance causes loss of vWF and factor VIII |
|
|
Term
| signs of the 4 types of vWF disease |
|
Definition
1. not clinically significant
2a. qualititive vWF deficiency
2b. mild thrombocytopenia
3. resembles hemophelia |
|
|
Term
|
Definition
spontaneous bleeding from mucous membranes, wounds, menorrhagia
prolonged bleeding time |
|
|
Term
| vWF disease: bleeding time, PTT, PT, other indicating tests 2 |
|
Definition
increased bleeding time
increased PTT (vWF stabilizes VIII)
normal PT
abnormal risocertin test
decreased VIII |
|
|
Term
| why is risocertin associated with vWF |
|
Definition
| pormotes vWF platelet agglutination, binding of vWF to GP1b |
|
|
Term
|
Definition
desmopressin
increases release of vWF from weible palade bodies in endothelial cells |
|
|
Term
| what are the categories of causes of DIC 15 |
|
Definition
obstretic complications
sepsis
neoplasm
endothelial injury
rattlesnake bite
hemangioma
vasculitis
aortic aneurysm
liver disease
heat stroke
burn
surgery
shock
trauma
acute intravascular hemolysis |
|
|
Term
| why do obstretic complicatins cause DIC, give 5 examples |
|
Definition
amniotic fluid contains tissue thromboplastin and activates coagulation cascade
abruptoplacentae
retained dead featus
septic abruption
toxemia
amniotic fluid embolism |
|
|
Term
| why does sepsis cause DIC, give 7 examples |
|
Definition
microbe toxins induce tissue factor release from monocytes which releases IL1 and TNF which increase THROMBOMODULIN
GNB, GPB, meningococcemia, malaria, RMSF, histoplasmosis, aspergillosis |
|
|
Term
| why does neoplasm cause DIC, give 5 examples |
|
Definition
express tissue factor III
pancreas, prostate, lung, stomach, acute promyelocytic leuemia |
|
|
Term
| what can cause widespread endothelial injury causing DIC 5 |
|
Definition
SLE
heat stroke
burn injury
meningitis
rickettsiae |
|
|
Term
| why does rattlesnake venom cause DIC |
|
Definition
|
|
Term
| why does traima cause DIC |
|
Definition
| brain releases phospholipids and fat and activate intrinsic cascade |
|
|
Term
| explain the MOA of DIC causing complications 3 |
|
Definition
widespread damage causes widespread microthrombi and FIBRIN DEPOSITION which causes ischemia and infarction
consumptive coagulopathy: formation of thrombi uses platelets, factors, fibrin leading to BLEEDING DIATHESIS
MICROANGIOPATHIC HEMOLYTIC ANEMIA: hemolysis of RBC in vessel narrowings |
|
|
Term
|
Definition
bleeding: prolonged, post-partum, petechiae/ecchymosis, GI/GU hemorrhages
thrombosis: abnormal clotting in microcirculation (minimal symptoms)
systemic: shock, acute real failure, dyspena, cyanosis, convulsions, coma
WATERHOUSE FRIDERISCHEN SYNDROME: microinfarcts in brain, neurological signs
SEEHAN POST PARTUM PITUITARY NECROSIS: eclampsia is hypercoagulable state and leads to thrombi in placenta, liver, kidney, brain and PITUITARY |
|
|
Term
| DIC: RBC changes, platelet count, PT, PTT, fibrinogen levels |
|
Definition
MICROANGIOPATHIC HEMOLYTIC ANEMIA: RBC sheared by thrombi
THROMBOCYTOPENIA
INCREASED PT AND PTT: consumed by all factors
decreased fibrinogen: busy working on thrombi
elevated D-dimers (fibrin split products): removal of clot after healing causes lysis of cross linked fibrin released D-dimers |
|
|
Term
|
Definition
|
|
Term
| cause of heparin induced thrombocytopenia |
|
Definition
| after 1-2wk heparin forms complex on platelet factor IV and body forms IgG autoantibodies that cause consumption of platelets by spleen |
|
|
Term
| what are the two types of HIT and what is the difference |
|
Definition
HIT1: platelets decrease due to non-immunologic mechanisms limited to 2-4d
HIT2: becomes life threatning(white clot syndrome) lasts 6-12d |
|
|
Term
|
Definition
platelet destruction causes fragmented platelets to enter circulation and allow thrombosis
arterial and venous thrombosis |
|
|
Term
|
Definition
DC heparin
begin lepirudin or warfarin
reoccurs with heparin use |
|
|
Term
| what 5 things does the endothelial cells release/have that do fibrinolysis |
|
Definition
t-PA/plasminogen
heparin like proteoglycans
prostacyclin
transmural negative charge
protein C activation |
|
|
Term
|
Definition
| turns plasminogen into plasmin |
|
|
Term
|
Definition
cleaves fibrin and serum fibrinogen
destories coagulation factors
blocks platelet aggregation |
|
|
Term
| what deactivates plasmin 3 |
|
Definition
a2 antiplasmin: degrades plasminogen when plasmin is unbound
PA-1 and urokinase tissue plasminogen activator (sCU-PA): endothelium degrades tPA |
|
|
Term
| what does prostacyclin do |
|
Definition
platelet inhibitor
binds and increases cAMP > activats phospholipase A2 > cyclooxygenase > prostacyclin synthase activated on membrane lipids > prostacyclin |
|
|
Term
| how does the transmural negative charge of entothelial cells affect clotting |
|
Definition
| helps fibrinolysis by preventing adhesion of platelets |
|
|
Term
| proteiin C activation role in clotting |
|
Definition
| degrades coagulation factors (thrombin modification with thrombomodulin) |
|
|
Term
| what are 2 diseases that affect fibrinolysis |
|
Definition
radial prostectomy
liver cirrhosis |
|
|
Term
| how does radial prostectomy affect clotting |
|
Definition
| release of urokinase in procedure activates plasmin which breaks down clotting factors and fibrinogen causing bleeding |
|
|
Term
| how does liver cirrhosis affect clotting |
|
Definition
| a2 antiplasmin is not produced and plasmin breaks down clotting factors and fibrinogen causing bleeding |
|
|
Term
| tx of liver cirrhosis decreased a2-antiplasmin |
|
Definition
| aminocaproic acid: blocks activation of plasminogen |
|
|
Term
| signs of liver cirrhosis decreased a2-antiplasmin |
|
Definition
|
|
Term
| liver cirrhosis decreased a2-antiplasmin: PT, PTT, bleeding time, platelet count, fibrinogen levels |
|
Definition
PT/PTT increased: clevage of clotting factors
increased bleeding time: inhibition of platelet aggregation
platelet count normal: not using them to make thrombi
increased fibrinogen split products but NO D-DIMER: there was no thrombus, no D dimer |
|
|
Term
|
Definition
| proliferation of T/B cell or precursors that spill into the blood |
|
|
Term
|
Definition
| blast cells unable to differentiate causing accumulation of blasts in marrow (>20% blasts required) |
|
|
Term
|
Definition
abrupt onset
fatigue, bleeding, fever infection
bone pain and tenderness
generalized lymphadenopathy
HSM
CNS signs: headache, vomiting, nerve palsies (meningeal spread) |
|
|
Term
|
Definition
most common: child t(12;21) TEL1-AML1 fusion
less common: adult transformation from CML t(9;22 phaldalphia) BCR-ABL increases TK activity |
|
|
Term
|
Definition
TdT
B Cell markers (CD 10, 19, 20)
blasts in peripherial blood
WBC <10,000 >100,000 or aleukemic
pancytopenia |
|
|
Term
| what does a ALL look like on a gemisa stain 6 |
|
Definition
lymphoblasts
coarse clumped chromatin
2 nucleoli
little agranular cytoplasm
glycogen granules
acid shiff positive |
|
|
Term
| treatment and prognosis of B-ALL |
|
Definition
excellent response to chemo
prophylaxis in CNS and scrotum needed
2-10y 80% chance with chemo
child form has better prognoses
better prognosis in females |
|
|
Term
|
Definition
activaating NOTCH1 mytation
NUP214-ABL fusion increases TK
hyperploidy |
|
|
Term
|
Definition
TdT
T cell markers CD2-8
blasts in peripherial blood
WBC <10,000 or >100,000 or aleukemic
pancytopenia |
|
|
Term
| what is the most common age and gender for T-ALL |
|
Definition
|
|
Term
| treatment and prognosis of T-ALL |
|
Definition
2-10yo male 80% chance success with chemo
not within range worse prognosis |
|
|
Term
| cause of CLL/SLL and 4 pathological changes in result |
|
Definition
high BCL2 stops B cell apotosis regulators
trisomy 21, deletion 11 or 12, or translocation
hypogammaglobulinemia: B cell can't make IgG
immune hemolytic anemia: if it does make Ig it is autoreactive to platelets and RBC
marrow destruction: tumor cells proliferation until displace normal marrow causeing pancytpenia
mutation of genoma transforms into diffuse large B cell lymphoma |
|
|
Term
|
Definition
presenting: fragility, weight loss, anorexia
lymphadenopathy: SLL
infections
anemia
bleeding
HSM |
|
|
Term
| lab markers for CLL/SLL 4 |
|
Definition
CLL WBC>4000 (not in SLL)
B cell markers CD 19, 20, 23
surface Ig heavy and light chains
CD5!!! (normally T cell, only other seen in mantle cell lymphoma) |
|
|
Term
| prognosis and complications of CLL |
|
Definition
median survival is 4-6y
slow growing
transformation into prolymphocytic leukemia, diffuse large B cell lymphoma |
|
|
Term
| cause of hairy cell leukemia |
|
Definition
mature B cell proliferation infiltrates spleen and marrow
due to activation mutation in serine TK BARF |
|
|
Term
| lab markers for hairy cell leukemia 5 |
|
Definition
hair cytoplasmic processes
TARP positive
B cell markers CD19, 20
surface Ig
markers CD11c CD103 |
|
|
Term
| clinical signs of hairy cell leukemia 4 |
|
Definition
splenomeagly massive (sequestration and extracellular hematopoesis)
older male
pancytopenia (B cells infiltrate marrow)
dry tap: fibrosis of marrow
NO lymphadrnopathy |
|
|
Term
| tx and prognosis of hairy cell leukemia |
|
Definition
2-CDA purine: adenosine deaminase inhibitor causes adenosine accumulation killing neoplastic B cells
excellent prognosis |
|
|
Term
| what are the 3 T cell non-hodgkins lymphomas |
|
Definition
cutaneous T cell lymphoma (mycosis fungoides)
adult T cell lymphocytic lymphomia
peripherial T cell lymphomia |
|
|
Term
| what are the 6 B cell non-hodgkins lymphomas |
|
Definition
follicular
mantle
extranodal/marginal
burkitt
diffuse large B cell
SLL |
|
|
Term
| cause of follicular lymphoma |
|
Definition
| t(14;18) BCL2-IgH causes BCL2 overexpression allowing more B cells to pass somatic hypermutationdue to apoptosis supression |
|
|
Term
| clinical signs of follicular lymphoma 2 |
|
Definition
patient >50yo
painless lymphadenopathy
usually generalized |
|
|
Term
| what do the follicles look like in follicular lymphoma |
|
Definition
hyperplasia in cortex that disrupts node architecture
no necrotic cells or tangible macrphages conducting apoptosis
small neoplastic cells with cleaved nuclei, infoldings, condensed chromatin
large non-neoplastic cells with vesicular chromatin and many nucleoli |
|
|
Term
| lab martkers for follicular lymphoma 3 |
|
Definition
B cell markers
BCL6 (germinal center marker)
BCL2 |
|
|
Term
| prognosis and complications of follicular lymphoma |
|
Definition
median survival 7-9y
no cure
pick up additional mutations and transform to diffuse large B cell lymphoma |
|
|
Term
| cause of mantle cell lymphoma |
|
Definition
t(11;14) cyclin D1 - IgH allows G1/S transition
more small B cells in mantle of lymph node |
|
|
Term
| clinical signs of mantle cell lymphoma |
|
Definition
lesions in marrow, blood, spleen, liver, node
lymphomatoid polyposis: lesion in GI that resembles polyp |
|
|
Term
| lab markers for mantle cell lymphoma 4 |
|
Definition
surface Ig
B cell markers
CD!! (normally in T cells, only other B cell vesion seen in CLL)
cyclin D1 |
|
|
Term
| prognosis of mantle cell lymphoma |
|
Definition
|
|
Term
| cause of extranodal/marginal lymphoma |
|
Definition
marginal zone is potential psace. enlarged by chronic inflammation.
H. pylori causes maltoma in marginal zone of GI malt nodes
t(11;18) MALT1-IPA2 |
|
|
Term
| where does extranodal/marginal lymphoma cause lesions 6 |
|
Definition
| stomach, salivary glands, bowel, lungs, orbit, breast |
|
|
Term
| tx and prognosis of extranodal/marginal lymphoma |
|
Definition
H. pylori caused can be tx with antibiotics
may need radiotherapy or local excision
if caused by translocation often wont respond |
|
|
Term
| cause of burkitt lymphoma and location of lesions |
|
Definition
t(8;14) IgH-cMYC increases B cell growth
latent EBV infection
african: 100% EBV caused, lesion in mandible or maxilla
sporatic: 25% EBV caused, lesion in bowel, retroperitoneal, ovary |
|
|
Term
| what do the cells look like on a smear in burkitt lymphoma |
|
Definition
B cells with round ovoid nuclei
lipid vacules
basophillia
STARRY SKY PATTERN: high rate of proliferation and apoptosis recruits macrophages that eat away a clear space |
|
|
Term
| lab markers in burkitt lymphoma |
|
Definition
surgace IgM
B cell markers
BCL8: germinal center marker
CD10: germinal center marker |
|
|
Term
| prognosis of burkitt lymphoma |
|
Definition
| fast growing, majority cured esp when young |
|
|
Term
| 2 cause of diffuse large B cell lymphoma |
|
Definition
BCL6 rerangement or mutation
t(14;18)IgH-BCL2 (transformed from follicular lymphoma) |
|
|
Term
| clinical signs of traditional diffuse large B cell lymphoma |
|
Definition
lesion in GI, brain, retroperitoneal
NOT commin in liver spleen or marrow |
|
|
Term
| what do the cells look like in diffuse large B cell lymphoma |
|
Definition
very large
diffuse growing in sheets (not follicle, mantle, etc)
poor differentiation (this is rare for lymphoid cells their either Blast or grown up) |
|
|
Term
| lab markers fo diffuse large B cell lymphoma 2 |
|
Definition
|
|
Term
| what are the three conditions that diffuse large B cell lymphoma is associated with, how does the disease present when combined with them |
|
Definition
lymphoma + EBV + immune comp (AIDS): transforms to clonal large B cell lymphoma in months
karposki + lymphoma: pleural, pericardial, and periosteal effusions
mediastina: in young females, spreads to abdominal viscera and CNS |
|
|
Term
| prognosis of diffuse large B cell lymphoma |
|
Definition
rapidly fatal if untrated
60% complete remission with chemo |
|
|
Term
| cause of mycosis fungoides |
|
Definition
| proliferation of CD4 T cells that invade skin |
|
|
Term
| clinical signs of mycosis fungoides |
|
Definition
| erythrodermic rash > plaque > tumor > sezary syndrome (hematogenous spread) |
|
|
Term
| lab markers for mycosis fungoides |
|
Definition
CD4T cells in epidermis and upper dermis make pautrier microabscesses
cerebri form nuclei when sezary |
|
|
Term
| prognosis of mycosis fungoides |
|
Definition
erythrodermic: years
plaque, tumor, sezary: 1-3y |
|
|
Term
| cause of adult T cell lymphocytic lymphoma |
|
Definition
| CD4 proliferatioon to to HTLV-1 |
|
|
Term
| clinical signs of adult T cell lymphocytic lymphoma |
|
Definition
rash
generalized lymphadenopathy
HSM
lytic bone lesions and hypercalcemia
transverse myelitis: demyelination of CNS |
|
|
Term
| lab markers of adult T cell lymphocytic lymphoma |
|
Definition
malignant lymphocytes in peripheral blood
CD4, CD25, IL2 |
|
|
Term
| prognosis adult T cell lymphocytic lymphoma |
|
Definition
median survival 8mo
15% have chronic course (same as mycosis fungoides) |
|
|
Term
| cause of peripheral T cell lymphoma |
|
Definition
| heterogenous group of tumors of functional T cells |
|
|
Term
| peripheral T cell lymphoma signs |
|
Definition
| disseminated disease due to tumor derived from inflammatory products |
|
|
Term
| prognosis peripheral T cell lymphoma |
|
Definition
agressive
responds poorly to therapy |
|
|
Term
| cause of hodgkins lymphoma |
|
Definition
| proliferation of reed sternburg cells from a germinal center B cell |
|
|
Term
| what do reed sternburg cells look like |
|
Definition
owl eye
multilobed nuclei
enclosed acidophilic nuclelous
clear zone around
(THESE DONT MAKE THE MASS they draw inflammatory cells which give it mass like shape) |
|
|
Term
| signs of hodgkins lymphoma |
|
Definition
stage IA-IIA: painless lymphadenopathy on single or chain of nodes
Stage IV+: fever chills, night sweats, puritis, anemia |
|
|
Term
| what are the 5 types of hodgkins lymphoma and their prognosis |
|
Definition
lymphocyte depleted - worse
lyphocyte - best
lymphocyte predominate
nodular sclerosis
mixed cellularity |
|
|
Term
| lymphocyte predominate hydgkins lymphoma: clinical and lab signs, prognosis |
|
Definition
cervical and axillary lymphadenopathy
small lymphocytes and histocytes
lymphocytic varient RS cells: have B cell markers
popcorn RS cells: puffy nuclei
excellent prognosis |
|
|
Term
| nodular sclerosis: cause, signs, lab markers, prognosis |
|
Definition
can be caused by EBV
in adolescent or young, supraclavicular cervical or mediastinal nodes
BANDS OF FIBROSIS in node
lacunar cells: RS sit in open spaces
no T/B cell markers
excellent prognosis |
|
|
Term
| mixed cellularity hodgkins lymphoma: cause, signs, lab markers |
|
Definition
EBV causes hyperactivity of NH-kB TG promoting B cell proliferation and protecting from apoptotic signals
>50yo, male, fever, night sweats, weight loss
heterogenous infiltrates, lymphocyes, eosinophils, plasma cells, histocytes
classic RS cells with no T/B cell markers |
|
|
Term
| cause of multiple myeloma |
|
Definition
proliferation of plasma cells in bome marrow that secrete complete or partial Ig (called M proteins)
due to cyclin D1, cyclin D3, FGF receptor 3, or MyC translocation next to IgH
fibroblasts and macrophages produce excess IL-6 to support the tumor |
|
|
Term
| 6 effects of multiple myeloma and why |
|
Definition
bone pain and hypercalcemia: tumor secretes IL-1, TNF, IL-7 which singals RANK and causes osteoclast bone reabsorption causing punched out lytic lesions on X ray and increased risk for fracture
immune supression: plasma cells secrete dysfunction Ig increasing risk for infection
renal dysfunction: dysfunctional Ig are toxic to the DCT and CD and make linear amyloid deposits of bence jones proteins allowing for bacterial pyelonephritis, metastastic calcification, and renal failure
hyperviscosity due to increased dysfunctional Ig in blood
reticuloendothelial take over: dysfunctional plasma cells take over spleen, marrow, kidney, liver, lungs, nodes, soft tissue (may turn into leukemia) causing pancytpenia |
|
|
Term
| lab markers of multiple myeloma |
|
Definition
increased IL-6
M proteins: usually IgG (some IgA or M)
Ig parts usually free light chains |
|
|
Term
| prognosis of multiple myeolma |
|
Definition
|
|
Term
| solitary/localized plasmacytoma: clinical signs, prognosis |
|
Definition
solitary skeletal or soft tissue tumor that makes M proteins
5-10y progression to multiple myeloma
often cured by local resection |
|
|
Term
| monoclonal gamopathy of undetermined significance: cause, clinical signs, prognosis |
|
Definition
cells have same translocations as multiple myeloma. pt has M proteins in serum and but no lytic lesion or other symptoms
develop into multiple myeloma at rate of 1% per year |
|
|
Term
| lymphoblastic lymphoma: cause, presentation |
|
Definition
B cell lymphoma makes IgM
generalized lymphadenopathy
waldenstorm macroglobulinemia: hyperviscosity causes visual impairment due to hemorrhage and exudates, neurological issues, bleeding, and cryoglobulinemia |
|
|
Term
| lab markers of lymphoblastic lymphoma |
|
Definition
B cells with mixture of types: small, paraneoplastic, normal
behave like indolent B celll lymphoma in marrow, nodes, spleen
produce IgM with normal heavy/light chains (NO bence jones)
hyperviscosity |
|
|
Term
| prognosis of lymphoblastic lymphoma |
|
Definition
|
|
Term
| heavy chain diseasse: cause, presentation |
|
Definition
varient of multiple myleoma
IgA: in lymphoid tissue, SI, respiratory
OR
IgG: in nodes, liver, spleen (HSM) |
|
|
Term
| primary immunocyte associtated amyloidosis: cause, presentation |
|
Definition
varient of multiple myeloma that only makes light chain
causes amyloid deposits |
|
|
Term
| what are the 2 leukopenias, which is most common, how many cells |
|
Definition
neutropenia/agranulocytosis <1000 cells/ul
lymphopenia (T/B cells) - less common |
|
|
Term
|
Definition
inadequate or ineffective granulopoiesis
increased grnulocytosis removal or destruction |
|
|
Term
| what are 3 causes of neutropenia caused by inadequate or ineffective granulopoiesis |
|
Definition
aplastic anemia: marrow failure
leukemia: marrow replaced by tumor
chemotherapy drugs |
|
|
Term
| what are 3 causes of neutropenia caused by increased granulocyte removal or destruction |
|
Definition
immune mediated injury: drug, autoimmune
splenic sequesteration: enlarged spleen, hypersplenism
infection: increased peropherial utilization pulls PMN into tissues |
|
|
Term
| 3 signs of neutropenia in patient |
|
Definition
susceptibility to bacterial and fungal infection
malase, weakness, fatigue
chills, fever
agranulocytic angina: ulceration of mucosa of mouth and pharynx |
|
|
Term
| what are 2 possible changes in the marrow in neutropenia and why |
|
Definition
marrow hypercellularity: when excess destruction or ineffective granulopoiesis
marrow hypocellularity: when drugs or autoimmune supressed granulocytopoesis |
|
|
Term
| treatment of neutropenia, why |
|
Definition
GMCSF, GCSF
stimulates neutrophil production in marrow |
|
|
Term
| what are 5 types of leukocytosis, how many cells |
|
Definition
WBC >10K
neutrophilic leukocytosis
reactive leukocytosis
monocytosis
eosinophilia
basophilia |
|
|
Term
| why is normal leukocytosis confused with leukemia |
|
Definition
| when WBC get so high they look like leukemia (often in kids with acute viral infection) called leukemoid reaction |
|
|
Term
| causes of neutrophilic leukocytosis 3, and some examples of each |
|
Definition
bacterial infection: esp pyogenic
tissue necrosis: MI, burns
high cortisol |
|
|
Term
| why does cortisol cause increase in neutrophils |
|
Definition
| cortisol disrupts adhesion of PMN increasing circulation of marginated pool |
|
|
Term
| 2 lab signs of neutrophilic leukocytosis |
|
Definition
immature neutrophils (shift left)
cells have fewer Fc (CD16) receptors (cannot recognize Ig as well) |
|
|
Term
| 2 causes of reactive leukocytosis |
|
Definition
viral infections
borditella pertussis |
|
|
Term
| why does boretella pertussis cause leukocytosis and not neutrophilia |
|
Definition
| bordatella blocks lymphcotyes from leaving blood to enter node for further development |
|
|
Term
|
Definition
saliva
normally in teenagers |
|
|
Term
| what are the risk factors for mono 2 |
|
Definition
unable to control infected B cells...
marrow and organ transplant patients: post transplant lymphoproliferative disorder
x-linked lymphoproliferative disorder |
|
|
Term
| when EBV gets in the body where does it go 3 |
|
Definition
| oropharynx, liver, B cells |
|
|
Term
| when EBV invades B cells what does it do in there 2 |
|
Definition
lytic: replication and release of virons
latent: polyclonal activation and proliferation, Ab secretion (heterophil anti-sheep red cell Ab) |
|
|
Term
| when EBV affects T cells what does it do |
|
Definition
| causes circulating atypical lymphocytes (not the main infection point, response to infection) |
|
|
Term
| what is the body's response to EBV at the location of initial infection 5 |
|
Definition
oropharynx: sore throat
liver: hepatitis, rarley jaundice
B cells: fever, lymphadenopathy (post. cervical, axilla, groin) |
|
|
Term
| what do the atypical T cells in EBV look like 4 |
|
Definition
large nuclei
abundent cytoplasm
oval nuclei
few granules |
|
|
Term
| why do people with EBV have splenomeagly |
|
Definition
white pulp (lymphoid T cell area peri-lymphatic sheath) expansion due to atypical lymphocytes
spleen in fragile and prone to rupture |
|
|
Term
|
Definition
increased liver enzymes
monospot test: detect IgM heterophile antibodies (positive within 1wk)
definitive testing: EBV viral capsid antigen |
|
|
Term
|
Definition
| none, normally self limiting in 2-6wk |
|
|
Term
|
Definition
hepatic dysfunction: jaundice, liver failure
splenic rupture: avoid sports to 1y
rash if exposed to penicillin
immune compormised allows uninhibited B cell proliferation that transforms into monoclonal B cell lymphoma |
|
|
Term
| causes of monocytosis 3, and some examples of each |
|
Definition
chronic infection: TB, endocarditis, rickettsia, malaria
collagen vascular disease: SLE
inflammatory bowel disease: ulcerative colitis |
|
|
Term
| causes of eosinophilis causes 9 |
|
Definition
asthma
hay fever
vasculitis
allergic reaction
phemigus
dermatitis
herpatiforms
parasites
drug reactions
malignancies: hodgkin lymphoma (increased IL-5) |
|
|
Term
|
Definition
|
|
Term
| what is the typical presentation of X-linked lymphoproliferative disorder, why is it a concern |
|
Definition
EBV can be fatal
usually young male |
|
|
Term
| acute non-specific lymphadenitis: location, cause |
|
Definition
group of nodes or generalized nodes
infection (systemic or localized) |
|
|
Term
| acute non-specific lymphadenitis: node color, shape, internal changes, cells |
|
Definition
swollen red-gray TENDER engourged nodes
large germinal center
central follicle necrosis and abscess when infection severe (may cause overlying skin reaction)
neutrophils: when caused by pyogenic organism |
|
|
Term
| chronic non-specific lymphadenitis: 3 types |
|
Definition
follicular hyperplasia
paracortical hyperplasia
sinus histocytes |
|
|
Term
| cellular changes in chronic non-specific lymphadenitis |
|
Definition
B cell activation causes follicular/germinal center reaction
node archecture preserved
variable germinal center size
T cells, B cells, tangible body macrophages appear |
|
|
Term
| cause of chronic non-specific lymphadenitis 3 |
|
Definition
inflammation or infection...
RA, early HIB, toxoplasmosis |
|
|
Term
| cellular changes in paracortical hyperplasia |
|
Definition
| immune reaction causes T cell activation |
|
|
Term
| 3 causes of paracortical hyperplasia |
|
Definition
viral infection
post-vaccination
drugs |
|
|
Term
| cellular changes in sinus histocytes 3 |
|
Definition
distension and prominence of sinusoids
infiltrated by macrophages
hypertrophy of lining of endothelial cells |
|
|
Term
| cause of sinus histocytes |
|
Definition
|
|
Term
| cause of cat scratch disease |
|
Definition
bartonella henslae infection via feline scratch
usually <18yo |
|
|
Term
| common signs of cat scratch disease, timing of signs, location |
|
Definition
regional lymphadenopathy: usually neck of axilla
2wk after scratch, lasts 2-4wks |
|
|
Term
| 3 rare signs of cat scratch disease |
|
Definition
encephalitis
osteomyelitis
thrombocytopenia |
|
|
Term
| what would a bopisy show in cat scratch disease, how do we visualize it |
|
Definition
silver stain
irregular stellate necrotizing granulomas: sarcoid like granulomas with neutrophils and central necrosis |
|
|
Term
| why does cat scratch disease get sent for biopsy analyzation so often |
|
Definition
| lymphoma is common in kids so need to be cautious, biopsy will clearly distinguish |
|
|
Term
|
Definition
hypospadius: urethra too high
epispadius: urethra too low |
|
|
Term
|
Definition
| genital tubrical (forms glans) failure to position |
|
|
Term
|
Definition
| urethra fold (seals penis as grows out) fails to seal |
|
|
Term
| complications of epispadius and hypospaius (2 associated contisions, 1 complication) |
|
Definition
associated with other genital abnormalities: inguinal hernia, undescended testis
may constrict urethral oriface |
|
|
Term
| 3 inflammatory conditions of penis |
|
Definition
inflammations blantitis/blantophosphitis
inflammations phimosis
inflammations paraphimosis |
|
|
Term
| inflammations blantitis/blanoposhitis: location |
|
Definition
blantitis: glans
blanoposthitis: glans and prepuce |
|
|
Term
| inflammations blantitis/blanoposhitis: cause |
|
Definition
| under foreskin smegma (sweat, debris, epithelial cells) build up and allow infection |
|
|
Term
| inflammations blantitis/blanoposhitis: 4 infections |
|
Definition
candidia
anaerobic
garderella
pyogenic bacteria |
|
|
Term
| inflammations blantitis/blanoposhitis: signs 4 |
|
Definition
distal penis red
swollen
tender
purulent discharge |
|
|
Term
| inflammations phimosis: cause |
|
Definition
| secondary to blanophosphitis causes scaring and adhesions of foreskin to glans |
|
|
Term
| inflammations phimosis: signs 3 |
|
Definition
cannot retract foreskin
increased smegma
smegma accumulation increases risk for carcinoma risk |
|
|
Term
| inflammation paraphimosis: cause, complication |
|
Definition
inflammation and swelling causes forcable retraction of foreskin
severe cases have urinary retention |
|
|
Term
| what is the most common proliferation in penile neoplasm |
|
Definition
|
|
Term
| most common age of penile neoplasm |
|
Definition
|
|
Term
| 5 risk factors for penile neoplasm |
|
Definition
phimosis
poor hygiene
HPV 16/18
smoking
lack of circumcusion |
|
|
Term
| 3 carcinoma in situ penile neoplasm |
|
Definition
bowen disease
erythroplasia of queryat
bowenoid papulosis |
|
|
Term
| bowen disease: appearance, location, age, risk |
|
Definition
glossy, solitary, white, plaque
SHAFT of penis
>30yo
1/3 visceral cancer risk |
|
|
Term
| erythroplasia of queryat: origin, appearance, location |
|
Definition
varient of Bowen disease
red plaque
GLANS penis and other mucosa |
|
|
Term
| bowenoid papulosis: cause, location, age, risk, prognosis |
|
Definition
HPV16
penile SHAFT
younger than bowens disease
almost never invasive carcinoma
may spontaneously regress |
|
|
Term
| penile squamous cell carcinoma: high risk population (age, ethnicity, other) |
|
Definition
<1% of all cancer
20% of males in asia, S. america, africa)
40-70yo
rare in uncircumcised males |
|
|
Term
| why is penile squamous cell carcinoma rare in uncircumcised males 2 |
|
Definition
better hygiene
decreased HPV risk |
|
|
Term
| penile squamous cell carcinoma: prognosis, metastasis locations |
|
Definition
slow growing, locally invasive
early metastasis: inguinal and iliac nodes (25% chance 5y survuval)
late metastasis: widespread (uncommon, 66% chance 5y survuval) |
|
|
Term
| penile squamous cell carcinoma: 2 types, prognosis of each |
|
Definition
keratinizing squamous cell carcinoma (normal penile SCC prognosis depending on metastasis timing)
verrucous carcinoma: papillary variant, locally invasive, no metastasis |
|
|
Term
| penile squamous cell caricnoma: clinical signs, color, lesion type, location |
|
Definition
not painful unless ulcerates (means its infiltrating) and develops irregular margins, bleeding, and infection
glans or pupuce
gray, crusted, papular |
|
|
Term
| explain the normal timeline of testis descent |
|
Definition
3mo gestation: abdomen to pelvis
last 2mo gestation: descend through inguinal canal to scrotum (may not be complete at birth) |
|
|
Term
| cause of cryptoorchidism, location |
|
Definition
complete or incomplete failure of testicular descent into scrotal sac
usually right testis
25% bilateral |
|
|
Term
| how is cryptoorchism diagnosed |
|
Definition
| determine testies location AFTER 1YO (wait until descent complete) |
|
|
Term
| complicatins of cryptoorchidism 5 |
|
Definition
testicular atrophy
haylinization by puberty
sterility
focal intratubular germ cell neoplasia (4x risk of cancer) |
|
|
Term
| associated conditions with cryptoorchidism 3 |
|
Definition
normally isolated
hypospadius
prader willi |
|
|
Term
| most common cause of epididmitis/orchitis in kids 2 |
|
Definition
gram negative bacteria
congenital abnormalities |
|
|
Term
| most common cause of epididmitis/orchitis in sexually active men <35yo 2 |
|
Definition
|
|
Term
| most common cause of epididmitis/orchitis in men >35yo 2 |
|
Definition
|
|
Term
| most common pathological cause of epididmitis/orchitis 3 |
|
Definition
cystitis, genitorostatis, urethritis
spread to testis via vasdeferens, spermatic cord, lymphatics |
|
|
Term
| epididmitis/orchitis: clinical signs 5 |
|
Definition
hx urethritis
pyuria
tenderness
swelling
age <35 |
|
|
Term
| epididmitis/orchitis: complications |
|
Definition
| sterility if not resolved in time |
|
|
Term
| gonorrhea: pathway of infections, complications 3 |
|
Definition
| urethra > prostate > seminal vesicles > epididmitis > epididmis abscess > testis > supurrative orchitis |
|
|
Term
| mumps: epidemology, timeline, effects 5 |
|
Definition
20-30% of post-puberty males infected
1 week after parotid swelling
seminiferous epithelium loss
tubular atrophy
necrosis
fibrosis
sterility |
|
|
Term
| TB: infection location 4, 2 complications |
|
Definition
epididmis > prostate, testis, seminal vesicles
caseating granulomas
necrosis |
|
|
Term
| 7 causes of testicular atrophy |
|
Definition
progressive athlerosclerotic narrowing of blood supply with age
end stage inflammatory orchitis
cryptoorchidism
hypopituitarism
malnnutrition/cachexia
irridation
prolonged female sex hormones |
|
|
Term
| testicular torsion: physiology |
|
Definition
| twisting of spermatic cord cuts off VENOUS supply but NOT ARTERY causing HEMORRHAGIC INFARCT |
|
|
Term
| causes of neonatal testicular torsion 2 |
|
Definition
in utero
at birth
NOT ANATOMICAL DEFECT |
|
|
Term
| causes of adult testicular torsion |
|
Definition
bilateral anatomical defect (bell clapper abnormality: testis not connected to inner scrotum)
associated with higher morbitity |
|
|
Term
| testicular torsion: 5 signs |
|
Definition
sudden pain
elevated testicle
absent cremasteric reflex
>20yo
congestion
extravasion of blood |
|
|
Term
| prognosis of testicular torsion |
|
Definition
UROLOGIC EMERGENCY
resolve within 6h |
|
|
Term
| variocele: cause, location, why |
|
Definition
dilation in vein within SPERMATIC CORD due to inadequate valves in vein
if rapidly developing is caused by renal cell carcinoma
usually in left testicle (more vesicles between it and IVC) |
|
|
Term
| variocele: signs, complications |
|
Definition
infertility in affected testicle
tortous mass on scrotal surface |
|
|
Term
| hydrocele: cause in infant |
|
Definition
| incomplete closure of TUNICA VAGINALIS allows connection to peritoneium and allows SEROUS FLUID in enlarging scrotal sac |
|
|
Term
| hydrocele: cause in adult |
|
Definition
| blocked lymph drainage due to idiopathic, infection, tumor, filarias |
|
|
Term
|
Definition
transillumination: serious fluid
none: lymph or blood (hematocele) |
|
|
Term
| what type of cancer is associated with chimney sweeps |
|
Definition
| squamous cell carcinoma of scrotum |
|
|
Term
| what is the cause of most scrotal SCC |
|
Definition
95% come from germ cells and are malignant and agressive
5% from sex cord or stroma and are benign but secrete steroids |
|
|
Term
| how does a testicle SCC normaly feel to the patient |
|
Definition
|
|
Term
| his is a testicle SCC diagnosed |
|
Definition
| it isnt. if there is a mass just remove it. biopsy could seed scrotum |
|
|
Term
| 6 risk factors for scrotal SCC |
|
Definition
cryptoorchidism
family or selx hx
testicular dysgenesis (feminization, klinefelter)
20-54yo
caicasian
NO ASSOCIATION WITH SMOKING, OBESITY, ETC |
|
|
Term
| is a germ cell neoplasm translucent |
|
Definition
|
|
Term
| what are the 7 germ cell neoplasm and the ages their seen in |
|
Definition
seminoma 40-50yo
spermatic seminoma elderly
embryonal carcinoma 20-30yo
yolk sac tumor 3yo
choriocarcinoma 20-30yo
teratoma all ages
mixed germ cell tumor 15-30yo |
|
|
Term
| seminoma: size, texture, edges, necrosis/hemorrhage, cell composition, cell type |
|
Definition
large 10x normal
soft
well demacrated
coagulation necrosis
glycogen rich cells
synctotrophoblast giant cells in 25% |
|
|
Term
| seminoma: diagnostic factors |
|
Definition
| placental alkaline phosphatase |
|
|
Term
| what tumor is identical to ovarian dysgenesis |
|
Definition
|
|
Term
|
Definition
VERY RADIOSENSITIVE
chemosensitive |
|
|
Term
| seminoma: prognosis, metastasis |
|
Definition
excellent prognosis
late metastasis: bular germ cell neoplasia |
|
|
Term
| serpmatocytic carcinoma: size, color, texture, cell composition |
|
Definition
larger than seminoma
pale gray
soft
mucoid cyst |
|
|
Term
| spermatocytic carcinoma: prgonosis, metastasis |
|
Definition
best prognosis
not associated with other neoplasia |
|
|
Term
| embryonal carcinoma: size, edges, hemorrhage/necrosis, cell orientation, cell type, cell composition |
|
Definition
smaller than seminoma
poor demacration
focal hemorrhage and necrosis
pleomorphic sheets and cords
synctal cells (often mixed with other germ cell tumors and glands)
basophilic cytoplasm |
|
|
Term
| embryonal carcinoma: diagnostic markers |
|
Definition
|
|
Term
|
Definition
|
|
Term
| embryonal carcinoma: prognosis, metastasis |
|
Definition
more agressive than seminoma
early metastasis |
|
|
Term
| what is the most common testicular tumor in kids |
|
Definition
|
|
Term
| yolk sac tumor: cell color, composition, edges, cell type, cell orientation |
|
Definition
yellow white
mucinous, mycrocyst, papillae
poorly differntated
cuboidal, columnar, schiller duval bodies, mixed embryonal carcinoma in adults
reticular network |
|
|
Term
| yolk sac tumor: diagnostic fctors |
|
Definition
|
|
Term
| schiller dubal bodies: where are they found, what are they |
|
Definition
in yolk sac tumor
resemble primitive glomeruli |
|
|
Term
| what is the only testicular tumor that does not cause any enlargement |
|
Definition
|
|
Term
| choriocarcinoma: size, hemorrhage/necrosis, cell types, location |
|
Definition
small
hemorrhage and necrosis
eosinophil synctal cells (dark nuclei), crytotrophoblasts, synchiotrophoblasts
cells programmed to find vessels so form around but not in proper villi |
|
|
Term
| choriocarcinoma: diagnostic markers |
|
Definition
|
|
Term
| signs choriocarcinoma, why |
|
Definition
| synctiotrophoblasts make H0hCG which cause hyperthyroid and gynecomastic becase they cause release of FSH/LH/TSH |
|
|
Term
|
Definition
| not radio or chemosensitive |
|
|
Term
| choriocarcinoma: prognosis, why |
|
Definition
rapid hematologic metastasis due to location around blood vessels
poor prognosis due to lack of radio or chemosensitivity |
|
|
Term
|
Definition
| neoplastic germ cells differentiate along somatic cell line |
|
|
Term
| teratoma: size, cell type, cell composition |
|
Definition
large
cartiladenous, mesenchymal (immature and mature)
cyst, solid |
|
|
Term
| associated clinical conditions to teratoma 4 |
|
Definition
| similar structures in thyroid, bronchi, intestine, nervous tissue |
|
|
Term
|
Definition
child and female benign
adult male malignant but local resection curative |
|
|
Term
|
Definition
| local resectiion curative |
|
|
Term
| what is the most common germ cell tumor |
|
Definition
| mixed is 60% of testicle germ cell neoplasms |
|
|
Term
| mixed germ cell tumor: most common composition 3 |
|
Definition
teratoma
embryonal carcinoma
yolk sac tumor |
|
|
Term
| mixed germ cell tumor: prognisus |
|
Definition
| prognsis based on worst prognsis cancer in the mix |
|
|
Term
| 3 sex cord stromal neoplasms, which is not a primary neoplasm, age ranges |
|
Definition
leyig cell tumors 20-60yo
sertoli cell tumors any
testicular lymphoma (not primary) >60yo |
|
|
Term
| normal function of leydig cells |
|
Definition
|
|
Term
| normal function of sertoli cells |
|
Definition
|
|
Term
| sign of leydig cell tumor in adult, why |
|
Definition
gynecomastia
tumor secretes androgens, estrogens, and corticosteroids |
|
|
Term
| sign of leydig cell tumor in kids, why |
|
Definition
| sexual percoity (early puberty due to hormone increase in androgens, estrogens, and corticosteroids) |
|
|
Term
| how can leydig cell tumors be identified |
|
Definition
| reinke crystals inside cells |
|
|
Term
| prognosis leydig cell tumor |
|
Definition
|
|
Term
| cause of sertoli cell tumors |
|
Definition
| masculinization or feminization causes decreased estrogen or andeogens |
|
|
Term
| signs of sertoli cell tumors 2 |
|
Definition
gynecomastia
usually asymptomatic |
|
|
Term
| prognosis of sertoli cell tumors |
|
Definition
|
|
Term
| what is the most common composition of testicular lymphoma |
|
Definition
| diffuse large cell non-hodgkins B cell lymphoma |
|
|
Term
| location of testicular lymphoma |
|
Definition
|
|
Term
| what maintains the function and composition of a normal prostate |
|
Definition
|
|
Term
| location of normal prostate |
|
Definition
anterior to rectum and under bladder
prosthetic urethra runs through it |
|
|
Term
|
Definition
glands
stroma (CT)
alkaline milky liquid added to seminal vesicle |
|
|
Term
| 2 non cancerous conditions of prostate |
|
Definition
prostatitis
nodular hyperplasia (BPH) |
|
|
Term
|
Definition
|
|
Term
| what is the most common cause of cancer in men and second most common cause of death in men |
|
Definition
|
|
Term
|
Definition
acute bactrial
chronic bacterial
chronic non-bacterial/chronic pelvic pain syndrome
asymptomatic inflammatory
granulomatous |
|
|
Term
| acute bacterial prostatitis: cause, 5 symptoms |
|
Definition
UTI microbes
fever, chills, dysuria, sepsis, tender boggy prostate |
|
|
Term
| chronic bacterial prostatitis: cause, 3 signs |
|
Definition
UTI microbes
recurrent UTI, low back pain, dysuria |
|
|
Term
| chronic non-bacterial prostatitis/chronic pelvic pain syndrome: cause |
|
Definition
who knows
prostatitis wo bacteria |
|
|
Term
| asymptomatic inflammatory prostatis: cause, diagnostic sign |
|
Definition
who knows, prostatitis wo bacteria
increased leukocytes in prostatic secretions |
|
|
Term
| granulomatous prostatis: cause |
|
Definition
| usually caused by BCG treating bladder cancer that mimics TB in prostate |
|
|
Term
| granulomatous prostatitis: diagnosis |
|
Definition
| ONLY prostatitis that needs biopsy |
|
|
Term
|
Definition
|
|
Term
|
Definition
| hyperplasia of stroma, epithelial cells, glands due to excess androgens, corpora amylacea secretion |
|
|
Term
|
Definition
large nodules in PERI-URETHRAL (CENTRAL, INNER TRANSITIONAL ZONE AROUND URETHRA) region of prostate
may project into bladder lumen making pedunculated mass |
|
|
Term
|
Definition
POST-VOID DRIBBLING
urethra obstruction
no increased cancer risk |
|
|
Term
| why does BPH cause urethra obstruction |
|
Definition
| may project into bladder lumen and form pedunculated mass causing ball valve urethra obstruction |
|
|
Term
| what are the 5 areas of alteration that cause adenocarcinoma |
|
Definition
androgens
hereditary
enivornment
acquired somatic mutations |
|
|
Term
| how do androgens play a role in adenocarcinoma |
|
Definition
| always develop after puberty, tumors resistant to anti-androgen therapy and have auto-activating androgen receptors |
|
|
Term
| how does hereditary play a role in adenocaricnoma |
|
Definition
increased risk in 1st degree relatives
uncommon in asians
common in AA due to MYC oncogene mutation
common >60yo |
|
|
Term
| what are SOMATIC mutations involved in adenocarcinoma |
|
Definition
mutations in TMPRSS2-RTS and PTEN tumor regulators
(dont forget about MYC oncogene mutation in AA which is NOT somatic) |
|
|
Term
| how does environment play a role in adenocarcinoma |
|
Definition
| diet, location, carcinogens have all shown some association |
|
|
Term
| signs of adenocarcinoma clinical localized disease 3 |
|
Definition
NO URINARY SYMPTOMS (LIKE POST VOID DRIBBLING)
lesion felt on rectal exam
positive PSA test
proliferation of peripherial glands |
|
|
Term
| signs of clinically advanced adenocarcinoma 2 |
|
Definition
back pain
OSTEOBLASTIC BONE DISEASE usually in lumbar (virtually diagnostic) |
|
|
Term
| how is adenocarcinoma diagnosed 4, what is the rating on each |
|
Definition
digital rectal exam: low sensitivity and specificity
transurethral ultrasound: poor specificity and sensitivity
transperitoneal/transrectal biopsy: required for conformation
prostate specific antigen (PSA) |
|
|
Term
|
Definition
2.5 ng/mL normal at 40
3/5 ng/mL normal at 50
4.5 ng/mL normal at 60
6.5 ng/mL normal at 70
>0.75ng/mL above normal is concern |
|
|
Term
| how specific is PSA to prostate cancer |
|
Definition
specific to prostate but not nessesciarly cancer
2/3 with cancer have increase
95% have increase when metastic
increases with nodular hyperplasia, prostatitis, insturmentation, ejaculation |
|
|
Term
| regular screening PSA isnt encouraged, why |
|
Definition
screening does not reduce mortality
it increased due to SE of cancer tx |
|
|
Term
| prognosis of adenocarcinoma: |
|
Definition
microscopic cancer(nodular hyperplasia) often found at biopsy and was asymptomatic
30% of men >50yo
70% of men >70yo will have occult prostate cancer |
|
|
Term
| what are the 3 categories of myeloid neoplasms |
|
Definition
acute myeloid leukemias
myeloproliferative disorders
myelodysplastic syndromes |
|
|
Term
| what are the 4 types of AML |
|
Definition
acute prolyelocytic leukemia
acute monocytic leukemia
acute megakaryoblastic leukemia
myelodysplastic syndrome |
|
|
Term
| what are the 4 myeloproliferative disorders |
|
Definition
chronic myeloid leukemia
polycythemia vera
primary myelofibrosis
essential thrombocythemia |
|
|
Term
| what is the general cellular process that leads to the symptoms in AML |
|
Definition
blast cells are unable to differentiate causing accumulation in the marrow
blasts crowd out normal hematopoiesis causing anemia, thrombocytopenia, and neutropenia. evuntally lead to marrow failure
blasts enter blood and and increase WBC count with large, immature (little cytoplasm) cells with punched out nucleolus |
|
|
Term
| what are the general clinical signs of AML 5 |
|
Definition
>55yo
abrupt onset: present within 3mo
depression of marrow function: fatigue, bleeding, fever, pallor, infection
bone pain and tenderness: marrow expansion, sub-periostral infiltration
if mass, will be discrete and granulocytic |
|
|
Term
| lab signs that distinguish AML 8 |
|
Definition
GRANULOCYTIC DIFFERENTIATION: MYELOPEROXIDASE AZUROPHILIC GRANULES IN AUER ROD CRYSTALIZATIONS
lysosomal non-specific esterase positive
blasts in peripherial blood and marro WBC<10000 or >100000
anemia, neutropenia, thrombocytopenia <100000 platelets0
acute leukemia: rare occurance where there is no blasts in blood
CD33: myeloid progenetor marker (also CD64, CD117, CD13-15) |
|
|
Term
| 4 characteristics of a AML on a wright gemisa stain |
|
Definition
fine chromatin
more cytoplasm
more granules
peroxidase positive |
|
|
Term
| cause of acute prolyeocytic leukemia |
|
Definition
| t(15l17) fuses RARA-PML blocking myeloid differentiation due to TF mutation |
|
|
Term
| TX prolyelocytic leukemia 3 |
|
Definition
take care of DIC first: EMERGENCY auer rods cause DIC
retinoic acid (vitA): overcomes block making neutrophiles and clearing tumor cells
chemo: use this too or pt will relapse |
|
|
Term
| prognosis of acute prolyelocytic leukemia |
|
Definition
|
|
Term
| how is acute monocytic leukemia diagnosed in the lab |
|
Definition
| positive for myeloperoxidase |
|
|
Term
| clinical signs of acute monocytic leukemia and why |
|
Definition
| proliferation of monoblasts that infiltrate gums causing ulceration and swelling |
|
|
Term
| 2 diagnostic criteria/associations for acute megakaryoblastic anemia |
|
Definition
lack myeloperoxidase
associated with down syndrom <5yo |
|
|
Term
| cause of myelodysplastic syndrome (AML) |
|
Definition
| exposure to alkylating agents or radiotherapy causes dysplasia of marrow cells, they are not permitted to enter the blood causing cytopenia and hypercellular marrow |
|
|
Term
| prognosis of myelodysplastic syndrome (AML) 2 |
|
Definition
death due to infection of bleeding
20% progress to acute leukemia |
|
|
Term
| general prognosis for AML 2 |
|
Definition
50% have long term disease
30% have long term disease with chemo
could benifit from marrow transplant |
|
|
Term
| risk factors for myelodysplastic syndrome 4 |
|
Definition
chemotherapy
alkylating agents
ionizing radiation
idiopathic |
|
|
Term
| explain the cellular changes in myelodysplastic syndrome |
|
Definition
| damage from risk factors causes loss of 5q or 7q which causes dysplasia in marrow cells and the marrow is replaced by transformed multipotential stems cells that can differentiate into RBC, granulocytes, platelets that are ineffective |
|
|
Term
| clinical signs of myelodysplastic syndrome 3 |
|
Definition
|
|
Term
| 3 signs in the marrow of myelodysplastic syndrome |
|
Definition
hyper or normocellular
megaloblastic erythroid precursors with Fe deposits in mitochondria (ringed sideroblasts)
granuulocyte precursors with abnormal granules |
|
|
Term
| prognosis of myelodysplastic syndrome 3 |
|
Definition
stem cells in marrow are unstable and could get mutations transforming it to AML
poor response to chemotherapy
mean survival 9-20mo |
|
|
Term
| general cause and cellular changes in chronic myeloproliferative disorders |
|
Definition
proliferation of myeloid progenitors that can terminally differentiate
involve activation of TK which generate signs mimicing hematopoietic GF |
|
|
Term
| clinical signs of chronic myeloproliferative disorders 4 |
|
Definition
later adult
high WBC
hypercellular marrow
may SEED nodes, liver, spleen (they make cancer too!) causing HSM and lymphadenopathy
hyperuricemia/gout: WBC break down products compete with uric acid
marrow fibrosis: marrow burns out, this causes transfer to acute leukemia |
|
|
Term
| cause of chronic myeloid leukemia |
|
Definition
95% t(9;22/phildalphia) fusion of BCR-ABL increases TK which mimics growth factors and causes a proliferation of GRANULOCYTES
5% subcytogenic BCR-ABL fusion along with other CH translocations |
|
|
Term
| clinical signs and stages 3 of CML |
|
Definition
pt 25-60yo
slow onset, non-specific symptoms
1. chronic: MASSIVE splenomeagly from extramedullary hematopoesis
2. accelerated: currently enlarging spleen, anemia, thrombocytopenia (only 50% have this phase)
3. transofrmation/blast crisis: transformation to acute leukemia (70%AML, 30%ALL) |
|
|
Term
|
Definition
leukocytosis >100000 (BASOPHILIA, neutrophils, myelocytes, metamyelocytes)
thrombocytosis
marrow hypercellularity
red pulp enlargement in spleen
leukocyte alkaline phosphatase LOW
t(9;22) ABL-BCR |
|
|
Term
|
Definition
marrow transplant: 70% curative, high risk
TK inhibitors |
|
|
Term
|
Definition
|
|
Term
| cause of polycythemia vera |
|
Definition
| JAK2 TK mutation activates EPO precursors increasing RBC |
|
|
Term
| clinical signs of polycythemia vera 17 |
|
Definition
middle age
slow onset
organ congestion and cyanosis: flushed face, HSM
extramedullary hematopoesis: HSM
thrombosis/infarct: heart, spleen, kidney
hemorrhage: GI, oropharynx, brain
headache
dizziness
hematemesis
melena
blurry vision
gout/hyperuricemia |
|
|
Term
| how do you distinguish polycythemia very from reactive and absolute polycythemia |
|
Definition
reactive: SaO2 increase
absolute: EPO increase
vera: no SaO2 increase, decreased EPO |
|
|
Term
| lab signs of polycythemia vera 4 |
|
Definition
decreased EPO
basophilia
HVT 60%
hypercellular marrow (progresses to spend with fibrosis leadiing to AML) |
|
|
Term
| tx of polycythemia vera 2 |
|
Definition
phelbotomy: decreased RBC mass
hydroxyurea |
|
|
Term
| prognosis of polycythemia vera 3 |
|
Definition
death w/o tx in 1y - vascular complications
10y survival - with phlebotomy
marrow failure --> AML |
|
|
Term
| cause of primary myelofibrosis |
|
Definition
JAK2 TK mutation increases megakaryocyte production
this crowds out and spends the marrow and shifts hematopoesis to spleen, liver, nodes causing HSM, anemia, thrombocytopenia, mild neutropenia |
|
|
Term
| clinical signs of primary myelofibrosis 5 |
|
Definition
HSM: once marrow fibrosed hematopoesis occurs in spleen, liver and nodes
increased infection, thrombosis, bleeding
hyperuricemia/gour |
|
|
Term
| why is there such fast fibrosis of marrow in primary myelofibrosis |
|
Definition
| megakaryocyte (platelet precursor) overproduction --> increased PDGF and TGFb which turn on fibroblasts in marrow |
|
|
Term
| lab signs in primary myelofibrosis marrow biopsy 6 |
|
Definition
fibroblasts
neoplastic megakaryocytes
abnormal RBC in odd shapes
leukoerythroblastosis
initially hypercellular with megakaryocytes
later hypocellular with fibrosis |
|
|
Term
| lab signs in blood biopsy in primary myelofibrosis |
|
Definition
abnormal RBC in odd shapes: PLIKILOCYTES, TEAR DROP RBC
immature WBC: leukoerythroblastosis, basophilia
large platelets: due to extramedullary hematopoesis
leukoerythroblastic smear |
|
|
Term
| what is leukoerythroblastosis |
|
Definition
| myelocytes and metamyelocytes |
|
|
Term
| what is a leukoerythroblastic smear |
|
Definition
| marrow contains reticulin gates to stop immature cells but in the spleen there are none so when it takes over hematopoesis (primary myelofibrosis) you seen myelocytes and metamyelocytes in the blood(nucleated RBC) |
|
|
Term
| primary myelofibrosis prognosis |
|
Definition
CML transformation
survive 4-5y
5-15% AML transformation |
|
|
Term
| what are langerhans cells, what do they do |
|
Definition
| immature dendritic skin cells derived from marrow monocytes that present antigen to nieve T cells |
|
|
Term
| cause of langerhans cells histocytosis |
|
Definition
| HLA-DR and CD1a cause neoplastic proliferation of langerhans cells that look histocytic rather than dendritic |
|
|
Term
| morphological characteristics of langerhans cells histocytosis 7 |
|
Definition
birbeck/tennis racket/HX bodies in cytoplasm (granules)
HLA-DR, MHC-II, CD1a, langerhin and S100 positive
vaculated cytoplasm |
|
|
Term
| what are the three langerhan cell histocytosis deasases |
|
Definition
letterer-swie disease (multisystem langerhands cell histocytosis/acute disseminated langerhans cell histocytosis)
eosinophilic granuloma (unifocal and multifocal langerhan cell histocytosis)
hans-schuller-christian disease |
|
|
Term
| clinical signs of letterer swie disease 10 |
|
Definition
<2yo
srborrhetic like erruptions
multifocal
if severe..
lymphadenopathy
pulmonary lesions
osteolytic bone lesions
HSM
marrow infiltration: pancythemia (recurrent otitis media and mastoiditis) |
|
|
Term
| tx and prognosis of letterer swie disease |
|
Definition
MALIGNANT
intense chemotherapy
rapidly fatal without tx
50% 5y survival with treatment |
|
|
Term
| cause of eosinophilic granuloma |
|
Definition
| expanding erosive accumulation of langerhans in medullary cavity of bones |
|
|
Term
| sings of unifocal eosinophilic granuloma 4 |
|
Definition
adolescent
skeletal lesions
asymptomatic (pain, tenderness)
pathological fracture: calvaria, ribs, femur |
|
|
Term
| prognosis of unifocal eosinophilic granuloma 2 |
|
Definition
heal spontaneously
cured with local excision or radiation |
|
|
Term
| signs of multifocal eosinophilic granuloma 10 |
|
Definition
in kids
multiple erosive bony lesions
infiltrate soft tissue: skin, lungs, stomach
diffuse skin erruptions: ears, scalp
ototis media, mastoiditis, URI
lymphadenopathy
HSM
50% involve posterior stalk and cause DI |
|
|
Term
| hans-schuller-christian disease clinical signs 4 |
|
Definition
lytic scalp defects
DI
exophalmos
>3yo |
|
|
Term
| hans-schuller-christian prognosis 2 |
|
Definition
spontaneous regression
often cured with chemo |
|
|
Term
| 8 things that cause massive (>1000g) splenomeagly |
|
Definition
myeloproliferative disorders: CML, primary myelofibrosis
chronic lymphocytic leukemia
hairy cell leukemia
lymphoma
malaria
gauchers
primary spleen tumor |
|
|
Term
| 13 things that cause moderate (500-1000g) splenomeagly |
|
Definition
portal HTN
splenic vein obstruction
acute leukemia
hereditary spherocytosis
thalassemia major
autoimmune hemolytic anemia
amyloidosis
neimann pick
metastic carcinoma/sarcoma
splenitis
TB
sarcoidosis
typhoid |
|
|
Term
| 6 things that cause mild (<500g) splenomeagly |
|
Definition
acute splenitis
mono
septicemia
intra abdominal infection
acute spleen congestion
SLE |
|
|
Term
|
Definition
enlarged spleen due to removal of excess circulation formed blood elements
most common and serious is platelets
second is RBC and PMN |
|
|
Term
| cause of thymic hyperplasia |
|
Definition
lymphoid follicles (germinal centers) in medulla with reactive B cells
present with myasthenia gravis, SLE, other autoimmune diseases |
|
|
Term
|
Definition
| neoplastic proliferation of thymic epithelial cells |
|
|
Term
| what does a thymoma look like: size, texture, color, location, cells around it |
|
Definition
firm and lobulated
white gray
15-20cm
encapsulated
in perithymic tissue
non-neoplastic thymocytes (immature T cells) by it |
|
|
Term
| what are the three types of thymoma, describe their cytological and biological agressiveness |
|
Definition
60% benigh: cytologically and biologicallt benign
20% malignant type I: cytologically benign, biologically malignant
5% malignant type II - cytologically and biologically malignant |
|
|
Term
| describe the cells in a benign thymoma |
|
Definition
medullary thymoma: spindled, elongated, medulla like
mixed thymoma: plump, round, cortical like |
|
|
Term
| where is the malignant 1 thymoma located |
|
Definition
locally invasive, occasionally metastasize
penetrates capsule and invades surroundiing vessels |
|
|
Term
| describe the epithelial cells of a malignant 1 thymoma |
|
Definition
abundant cytoplasm
round vasicular nuclei
some spindled epithelial cells |
|
|
Term
| what does a malignant 2 thymoma look like |
|
Definition
|
|
Term
| what tumors does malignant 2 thymoma behave like |
|
Definition
| squamous cell carcinoma or lymphoepithelioma like carcinoma |
|
|
Term
| what two cellular characteristics does malignant 2 thymoma have |
|
Definition
EBV genome included
many tymocytes around it |
|
|
Term
|
Definition
|
|
Term
| what is 85% of primary kidney tumors |
|
Definition
|
|
Term
| who gets renal carcinoma: gender, age |
|
Definition
|
|
Term
| pathology of renal carcinoma: tissue type, color, location, cell description |
|
Definition
growth of renal tubular cortex epithelium
well defined yellow orange mass on upper pole with processes into parenchyma
cells are vacoulated (lipid-laden) or solid |
|
|
Term
| 4 risk factors for renal cell carcinoma |
|
Definition
smoking
cadmium
dialysis acquired cysts
polycystic kidney disease |
|
|
Term
| 3 types of renal cell carcinoma, which is more common |
|
Definition
clear cell 80%
papillary renal cell carcinoma
chromophobe renal carcinoma |
|
|
Term
| how does sporadic clear cell carcinoma present |
|
Definition
| single tumor on upper pole of kidney |
|
|
Term
| cause of familial clear cell carcinoma |
|
Definition
loss of VHL tumor supressor on 3-25 increases IGF and HIF which increases VEGF and PGDF
associated with von hippel lindau |
|
|
Term
| what are the tumors of von hippel lindau |
|
Definition
| hemangioma of cerebellum, retina, other neoplasms |
|
|
Term
| presentation of familial clear cell carcinoma |
|
Definition
| hundreds of bilateral kidney masses |
|
|
Term
| clear cell renal carcinoma: grade, metastasis locations |
|
Definition
very invasive
often extends through calyces, pelviz, ureters, enters RENAL VEIN as far as right atrium
high incidence of metastasis: lung, bone, regional nodes |
|
|
Term
| signs of clear cell renal carcinoma: 4 |
|
Definition
classic triad: hematuria, palpable mass, flank pain
other symptoms depending on the paraneoplastic association |
|
|
Term
| what are the 4 paraneoplastic types of clar cell renal carcinoma and the outcome |
|
Definition
ACTH: cushing syndrome
PTH: hypercalcemia
renin: HTN
erythropoetin: polycythemia |
|
|
Term
| causes of papillary renal cell carcinoma |
|
Definition
familial or sporadic
CH7 trisomy causes amplification of MET (TK) causing growth of PCT epithelium |
|
|
Term
| papillary renal cell carcinoma: color, characteristics, location, |
|
Definition
multiple bilateral papillary growth pattern
less yellow (less lipid than clear cell), necrosis, hemorrhage, cystic |
|
|
Term
| chromophobe renal carcinoma cause |
|
Definition
| intercalated cells of the CD have lost many chromosomes (hypodiploidy) |
|
|
Term
| chromophobe renal carcinoma: color, appearance, location, size |
|
Definition
stain dark
tan brown
nuclei with halos of clear cytoplasm
macrovesicles |
|
|
Term
| what is the 3rd most common cancer in kids |
|
Definition
|
|
Term
| who gets wilms tumor: age |
|
Definition
|
|
Term
|
Definition
| deletion or mutation in WT1/WT2 on Ch11 |
|
|
Term
| 3 syndromes associated with wilms tumor |
|
Definition
WAGR syndrome
denys drash syndrome
beckwith weidmann syndrome |
|
|
Term
|
Definition
wilms tumor
aniridia: no iris
ganital abnormalities: hyposapdias, epispadius
mental retardation |
|
|
Term
| signs of denys drash syndrome 3 |
|
Definition
gonadal dysgenesis
renal abnormalities
wilms tumor |
|
|
Term
| cause of beckwith weidemann syndrome |
|
Definition
improper genomic imprinting: region Ch11 normally on paternal CH (maternal is silenced).
in disease maternal copy is not imprinted (is active) and IGF-2 is over produced |
|
|
Term
| signs of beckwith weidmann syndrome 2 |
|
Definition
wilms tumor
organomeagly: tongue, kidney, hemihyprotrophy (entire body segments) |
|
|
Term
| wilms tumor: cell type, color, characteristics, size |
|
Definition
blastoma (kidney mesoderm precursor cells) make primitive glomeruli, tubules, and stroma
well circumscribed, soft, large, tan/gray, focal hemorrhages/necrosis
"tumor makes its own kidney" |
|
|
Term
| clinical signs of wilms tumor 5 |
|
Definition
palpable abdominal mass,
abdominal pain, fever, hematuria, intestinal obstruction |
|
|
Term
|
Definition
| survival for 2y implies recovery is possible |
|
|
Term
| 5 congenital abnormalities of the ureters |
|
Definition
double ureters
ureteroprlvic junction obstruction
diverticula
hydroureter
megaloureter |
|
|
Term
| double ureter: define, complication |
|
Definition
derived from double or split ureteral bud
can cause obstruction of flow |
|
|
Term
| urteropelvic junction obstruction: who gets it, location |
|
Definition
usually in infant boy left ureter (most common cause of hydronephrosis in kids)
adults more common in women unilateral |
|
|
Term
| diverticula: cause, definition, complication |
|
Definition
congenital or acquired
saccular outpouching of ureteral wall that allows for stasis and infection |
|
|
Term
| hydroureter: cause, define |
|
Definition
congenital or acquired, may be nephrogenic defect in ureteral musculature innervation
dilation, elongation, and toursity of the ureters |
|
|
Term
| megaloureter: define, complications |
|
Definition
enlarged ureter due to functional deficit in ureteral muscle
hydronephrosis and decreased renal function |
|
|
Term
| what is the inflammation of the ureter called |
|
Definition
|
|
Term
|
Definition
usually component of UTI
ureteritis cystica: glandular metaplasia due to persistant infection/damage causing chronic inflammatory changes |
|
|
Term
| 4 obstructive lesions of the ureters |
|
Definition
hydronephrosis
hydroureter
pyelonephritis
sclerosing retroperitoneal fibrosis |
|
|
Term
| sclerosing retroperitoneal fibrosis: age, cellular changes, complications, disease associations |
|
Definition
middle aged
fibrous and inflammatory proliferation encases retroperitoneal structures causing obstructive hydronephrosis
ormond disease |
|
|
Term
| ormond disease: cause, location, triggers |
|
Definition
autoimmune reaction causes IgG4-secreting plasma cells to activate
often in pancreas and retroperitonium and salivary glands
triggers: drugs, malignancy, idiopathic |
|
|
Term
| 3 neoplasms of the ureter, are they benign or malignant |
|
Definition
benign primary neoplasias: fibroepithelial polyps, leiomyomas
primary malignant tumors: usually transitional cell carcinoma |
|
|
Term
| fibroepithelial polyps: appearance, locations 4 |
|
Definition
small mass projecting into lumen
more common in left ureter
also in bladder, pelvis, urethra |
|
|
Term
| primary malignant ureter tumors: location, age, cell type |
|
Definition
usually transitional cell carcinoma
simillar to those in renal pelvis, calyces, and bladder
50-60yo |
|
|
Term
| 5 congenital abnormalities of the bladder |
|
Definition
diverticula
exstrophy
vesicureteral reflux
congenital fistulas
persistant urachus |
|
|
Term
| diverticula: cause, changes in tissue |
|
Definition
congenital defect or persistant urethral obstruction (like BPH)
obstruction or increased vesicular pressure causes thickening of bladder wall and outpouching |
|
|
Term
| diverticula: clinical signs |
|
Definition
usually asymptomatic
if symptomatic means it made area of stasis and allowed for infection, caliculi, and carcinoma (gets through muscle wall fast)
predisposition to vexicuroetal reflux |
|
|
Term
|
Definition
| developmental failure causes bladder wall to flatten and protrude through abdomen wall |
|
|
Term
|
Definition
| chronic infection and inflammation, increased carcinoma risk (esp adenocarcinoma) |
|
|
Term
| complications of vesicoureteral reflux |
|
Definition
| predisposition to chronic pyelonephritis |
|
|
Term
| what is a congenital fistula |
|
Definition
| connection between bladder, vagina, rectum or uterus |
|
|
Term
| what is a persistant urachus |
|
Definition
fistulous urinary tract between bladder and umbilicus
allows for formation or urachal cysts which allow carcinoma and infection |
|
|
Term
| what are the 4 types of cystitis |
|
Definition
infectious
hemorrhagic
suppurative/ulcerative
chronic |
|
|
Term
| causes of infectious cystitis 4 |
|
Definition
e. coli
proteus
klevesebella
enterobacter |
|
|
Term
| caises of hemorrhagic cystitis |
|
Definition
cytotoxic antitumor trugs (cyclophosphamide)
bladder radiation
adenovirus |
|
|
Term
| cause of suppurative/ulcerative cystitis |
|
Definition
| ulceration and sloughing of large areas of mucosa |
|
|
Term
| cause of chronic cystitis |
|
Definition
| persistence of infection causes fibrous thickening of muscularis propria decreasing bladder elasticity |
|
|
Term
|
Definition
urinary frequency (15-20min)
abdominal pain: suprapubic
dysuria: pain with urination
systemic: fever, chills, malaise |
|
|
Term
| 3 types of non-infectious cystitis |
|
Definition
interstitial - possibly autoimmune
polyploid
malacoplakia |
|
|
Term
| signs of interstitial cystitis 7 |
|
Definition
| urgency, frequency, hematuria, dysuria, intermittent severe suprapublic pain, female, no bacterial infection |
|
|
Term
| tissue changes in interstitial cystitis 5 |
|
Definition
cytoscopic fissures
punctuate hemorrhages
hunner ulcers: mucosal ulcers
later: transmural fibrosis
later: contraction of all layers |
|
|
Term
| cause of polyploid cystitis, effect |
|
Definition
irritation of bladder mucosa due to injury (usually catheter)
uroepithelium is thrown into broad bulbous polyploid projections causing submucosal edema |
|
|
Term
| what does papillary urothelial carcinoma often look like |
|
Definition
|
|
Term
|
Definition
chronic infection (E. coli, proteus)
immune supression
macrophage defects (overload with bacterial products) |
|
|
Term
| location of malacoplakia 6 |
|
Definition
| colon, lungs, bone, kidney, prostate, epididumis |
|
|
Term
| malacoplakia: color, size, density, cell types |
|
Definition
macroscopic, soft, yellow, 3-4cm, mucosal plaques
macrophages stuffed with bacterial remnants
multinuclate giant cells
michaelis-gutman bodies: macrophage lysosomes with Ca deposits |
|
|
Term
| what are 4 metaplasias of the bladder |
|
Definition
cystitis glandularis
cystitis cystica
intestnal or colonic metaplasia
squamous metaplasia |
|
|
Term
| how is cystitis glandularis identified |
|
Definition
| brunn nest: lesion in bladder transitional epithelium grows down onto lamina propria and transforms into cuboidal or columnar epithelium |
|
|
Term
| what does cystitis cystica look like |
|
Definition
| cystic spaces lined by flattened urothelium |
|
|
Term
| intestinal or colonic metaplasia: how does this affect bladder |
|
Definition
| glblet cells resembling intestinal mucosa form in bladder |
|
|
Term
| cause of squamous metaplasia |
|
Definition
| thickening in response to injury |
|
|
Term
| bladder epithelium and mesenchyme tumors: prevelance, most common type, |
|
Definition
>57,000/y and increasing
usually transitional cell carcinoma
<5% true squamous cell carcinoma |
|
|
Term
| urethelial neoplasms: location, general changes |
|
Definition
benign to agressive, high risk of death
multifocal in any site with urothelium (renal pelvis to urethra)
keratinization, chronic irritation, infection |
|
|
Term
| two urethelial neoplasm precursor lesions |
|
Definition
non-invasive papillary tumor
carcinoma in situ |
|
|
Term
| cause of non-invasive papillary tumor |
|
Definition
| papillary urothelial hyperplasia |
|
|
Term
| urethelial neoplasm carcinoma in situ: cell type, different shapes and their prognosis |
|
Definition
cytologically malignant cells
flat urothelium
full thickness or urethrlium
pategoid spread: scattered malignant cells in urine often loose cohesiveness spread and seed |
|
|
Term
| 4 types of urethrlial neoplasm |
|
Definition
papilloma
non-invasive papillary urothelial neoplasm of low malignant potential (grade I urothelial carcinoma)
grade II urothelial carcinoma (low grade papillary urothelial carcinoma)
grade III urothelial carcinoma (high grade urothelial carcinoma) |
|
|
Term
| papillary urothelial carcinoma: who gets it, prevelance, shape, prognosis |
|
Definition
young patients
1% of bladder tumors
small finger like papillae identical to normal urothelium
does not progress to cancer, rarley reoccurs |
|
|
Term
| noninvasive papillary urothelial neoplasm of low malignant potential prognosis |
|
Definition
| rarley invasive, may reoccur after removal |
|
|
Term
| grade II urothelial carcinoma: appearance, prognosis |
|
Definition
rarley invasive
rarley life threatning
papillary exophytic growth with cellular arypica |
|
|
Term
| grade III urothelial carcinoma: two types, their prognosis |
|
Definition
papillary
flat: highly agressive, rapid progression |
|
|
Term
| squamous cell carcinoma of bladder: prevelance, cause, appearance, prognosis |
|
Definition
3-7% of US cancer
more frequent with schistomiasis endemics
cover large area of bladder
deeply invasive |
|
|
Term
|
Definition
| malignancy of gland cells (not normally in bladder so came) from urachius elements on bladder dome |
|
|
Term
| adenocarcinoma complications |
|
Definition
histologically identical to adenocarcinoma of the GI
rare varients include highly malignant signet ring cell carcinoma |
|
|
Term
|
Definition
| connects fetal duct to yolk sack allowing bladder to drain waste through dome of bladder to umbilical cord |
|
|
Term
| bladder cancer: age, gender, common cause |
|
Definition
more in men, industralized nation, urban
80% 50-70yo
usually not familian |
|
|
Term
| risk factors for bladder cancer |
|
Definition
#1 smoking
industrial: arylamines (B-naphyline) for 15-40y
schistoma hematobium endemics
long term analegesics
long term cyclophosphamide
radiation: years after exposure, other pelvic malignancies |
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|
Term
| what cancer does schistoma hematobium usually cause |
|
Definition
mostly SCC, rest TCC
increases keratinization |
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Term
| 4 steps in bladder neoplasm clinical course |
|
Definition
PAINLESS HEMATURIA: sometimes only sign
frequency, uregency, dysuria
external urethral oriface hydronephrosis or pyelonephritis
after excision: new tumors of high grade develope |
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|
Term
| what are the factors involved in progression free survival of bladder cancer |
|
Definition
early detection is key (catch in lamina propria)
grade, lamina propria invasion, associated CIS |
|
|
Term
| what is the worse type of bladder cancer |
|
Definition
|
|
Term
| where does bladder cancer metastasize locally 5 |
|
Definition
prostate, seminal vesicles, ureters, retroperitonieum
fistula to vagina or rectum |
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|
Term
| where does bladder cancer metastasize distandly and how |
|
Definition
| hematogenous dissemination (rare) to liver, lungs, marrow |
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|
Term
| what is the mainstay of diagnosis of bladder cancer |
|
Definition
|
|
Term
| what is the inflammation of urethra called, what are two tyopes |
|
Definition
| urethritis: gonoccal, non-gonoccal |
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Term
| cause of gonococcal urethritis |
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Definition
| early manifestation of N. gonococcus |
|
|
Term
| cause of non-conoccal urethritis |
|
Definition
| E. coli, GNB (coliforms), chalmydia |
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|
Term
| signs of non-gonoccal urethritis |
|
Definition
cystitis in females
prostatitis in males
reiter syndrome |
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|
Term
| 3 signs of reiter syndrome |
|
Definition
arthiritis
conjunctivitis
urethritis |
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|
Term
|
Definition
not a serious clinical problem in itself
local pain
itching
frequency |
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|
Term
|
Definition
urethral caruncle
primary carcinoma |
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|
Term
| urethral caruncle: size, location, color, cause |
|
Definition
| small, red mass on external urethral meatus in females due to chronic irritation of prolapsed urethra |
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|
Term
| urethral caruncle: who gets it |
|
Definition
any age
usually post menopause women |
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|
Term
| urethra caruncle: morphology |
|
Definition
friable
trauma causes ulceration and bleeding
may mimic carcinoma
small red mass |
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|
Term
| primary carcinoma of urethra: who gets it, associations |
|
Definition
usually advanced aged females
cancers also occur within prostatic urethra |
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