Term
| what causes right Hb/O2 association shift |
|
Definition
less saturated
acidosis, increased body temp, high altitude, exercise, increased CO2, 2,3-BPG |
|
|
Term
| what causes left Hb/O2 association shift |
|
Definition
more saturated
alkalosis, decreased body temp, low altitude, rest, decreased CO2, decreased 2,3-BPG, HbF |
|
|
Term
| when chronically anemic what does the body do to compensate |
|
Definition
| increased CO (SV, HR), right shift of Hb curve, increased 2,3-BPG, expansion of plasma volume |
|
|
Term
| when should a transfusion be given to an anemic patient |
|
Definition
|
|
Term
| Fe deficiency: ferritin, Fe, TBIC/transferrin, Fe:TBIC, RDW, blood smear |
|
Definition
ferritin low
Fe low
TBIC/transferrin high
Fe:TBIC <12% low
RDW high
hypochromic, target cells |
|
|
Term
| anemic of chronic disease: ferritin, Fe, TBIC/transferrin, Fe:TBIC, RDW, blood smear |
|
Definition
ferritin: normal high
FE low
TBIC:transferrin low
Fe:TBIC >18% normal
RDW normal
hypochromic |
|
|
Term
| thallasemia: ferritin, Fe, TBIC/transferrin, Fe:TBIC, RDW, blood smear |
|
Definition
ferritin normal
Fe normal high
TBIC/trans ferrin normal
Fe:TBIC normal
RDW normal high
target cells, stippled, darocytes |
|
|
Term
| sideroblastic anemia: ferritin, Fe, TBIC/transferrin, Fe:TBIC, RDW, blood smear |
|
Definition
ferritin high
Fe high
TBIC/transferring variable
Fe:TBIC normal
RDW high
ringed sideroblasts |
|
|
Term
| lead poisoning: ferritin, Fe, TBIC/transferrin, Fe:TBIC, RDW, blood smear |
|
Definition
ferritin normal
Fe normal high
TBIC/Transferrin normal
Fe:TBIC normal
RDW high
basophilic stippling, ringed sideroblasts |
|
|
Term
| causes of microcytic anemia |
|
Definition
copper deficiency
iron deficiency
lead poisoning
anemia of chronic disease
thallasemia
sideroblastic anemia |
|
|
Term
|
Definition
| pallor, fatigue, weakness, dyspnea, orthostatic hypotension, tachycardia, pica (ice, clay, dirt), restless legs, angular kyelitis, spooning of nails |
|
|
Term
| types signs and tx of a-thalassemia |
|
Definition
silent carrier (1 deletion): asymptomatic, normal Hct
trait/minor (2 deletion): mild anemia, no tx
HbH disease (3 deletion): excess b-globin join making HbH, shift left, hemolytic anemia, splenomeagly, transfusions
HbBart (4 deletion): fetus cannot make HbF (2a/2g) so make HbBart (4g) and shift left, hydrops fetalis, death shortly after birth |
|
|
Term
| risk factors for thallassemia |
|
Definition
a: african, asian
b: mediterranian, middle eastern, indian |
|
|
Term
| types signs and tx b-thalassemia |
|
Definition
minor (hetero): HbA2 (a/g) increase, asymptomatic, mild anemia, no tx
intermedia (homo): HbA2 and HbF increase, target cells, anemia, occasional transfusions
major (hetero): no b-globin, HbA2 and HbF increase, HSM, marrow distortion, failure to thrive, crew cut skull, target cells, frequent transfusions |
|
|
Term
|
Definition
defective heme synthesis
anemia, angina, pallor, dyspnea, tachypnea, increased PP, systolic murmur
10% develop acute leukemia |
|
|
Term
| sideroblastic anemia causes |
|
Definition
| chloramphenicol, isionazid, alcohol, lead, neoplasm sinc, copper deficiency, collagen vascular disease, hereditary |
|
|
Term
| sideroblastic anemia treatment |
|
Definition
hereditary: pyrodixine (B6) to normalize Hb concentration
acquired: remove cause, EPO supplement
both: phlebotomy, deferoxamine chelation |
|
|
Term
|
Definition
sideroblastic anemia
fatigue, weakness, cramping abdomen, joint pain, headache, short term memory loss, lead lines on gingiva, peripheral neuropathy
adult: EDTA, succimer
kid: EDTA, succimer, dimercaperol |
|
|
Term
| cause of anemia of chronic disease |
|
Definition
| release of inflammatory cytokines suppresses erythropoiesis trapping Fe in macrophages, decreases EPO production, increases hepcidin levels (inhibits Fe absorption and mobilization) |
|
|
Term
|
Definition
| neutropenia, thrombocytopenia, normochromic normocytic anemia, marrow biopsy with hypocellular marrow full of fat, no progenitor cells |
|
|
Term
|
Definition
sore inflammed tongue, lossitis
LE neutropathy, ataxia
diarrhea
dementia, systolic murmur, decreased DTR, urinary and fecal incontinence |
|
|
Term
| how can you tell B12 and folate deficiency apart |
|
Definition
levels may be normal even in deficiency
folate increases homocystine
B12 increases homocystine and methylmalonic acid |
|
|
Term
|
Definition
dose unlabeled B12 to saturate
dose radioactive B12 and measure in urine to see if absorbed
repeat but add intrinsic factor
pernicious anemia: add intrinsic factor will solve problem
malabsorption: adding intrinsic factor will not solve problem |
|
|
Term
| causes of folate deficiency |
|
Definition
| tea and toast diet, alcoholism, methotrexate, TMP-SMX, pheytoin, pregnancy, hemolysis, hemodialysis |
|
|
Term
|
Definition
| altered metabolism of plasma lipoproteins into their membranes alters RBC shape increasing RBC volume (macrocytic anemia) |
|
|
Term
| case of: schistocytes, spherocytes/helmets, burr/echinocytes, spur/acanthocytes, bite cells, sickle cells, heinz bodies |
|
Definition
schistocytes: intravascular hemolysis
spherocyte: extrevascular hemolysis
burr: regular spikes, uremia, hemolysis
spur: irregular spikes, abetalipoproteinemia
bite: G6PDH deficiency
sickle: sickel cell anemia
heinz; G6PDH deficiency |
|
|
Term
|
Definition
| binds hb and is removed in spleen, will be low in hemolytic anemia |
|
|
Term
|
Definition
| released from RBC, will be high in hemolytic anemia |
|
|
Term
| what is and cause of direct coombs |
|
Definition
reagent mixed with pt RBC, agglutination means RBC is coated with IgG and complement
warm or cold agglutinins |
|
|
Term
| what is and cause of indirect coombs |
|
Definition
mixed serum with type O RBC and them with coombs reagent, agglutination indicates anti-RBC Ab in serum
erythroblastosis fetalis |
|
|
Term
| causes of warm hemolytic anemia |
|
Definition
| lymphoma, CLL, cancer, SLE, a-methyldopa |
|
|
Term
| causes of cold hemolytic anemia |
|
Definition
|
|
Term
| pathogenesis of cold/warm hemolytic anemia |
|
Definition
warm: IgG, extravascular hemolysis
cold: IgM, intravascular hemolysis |
|
|
Term
| cause and signs of mechanical hemolysis |
|
Definition
turbulent flow through mechenical valves, microangiopathic hemolytic anemia extrahemolytic hemolysis, HTN, coagulation disorders
schistocytes, hemolytic anemia |
|
|
Term
| cause of sick cell anemia |
|
Definition
AR
HbA is replaced by HbS due to substitution of uncharged valine for negative glutamic acid in 6th position of B-globin chain
homozygous patients have sickling in low O2 conditions due to Hb polymerization that can obstruct small vessels leading to ischemia |
|
|
Term
|
Definition
high output CHF and CP
chronic hemolytic anemia
aplastic crises
jaundice and pallor
venous occlusion (lat malleoli)
autosplenectomy due to infarcts
pigmented gall stones
bone pain from crises
dactylitis
osteonecrosis (humeral/femoral head)
marrow hyperplasia (hair on end calvarium)
fish vertebrae: infarct in middle but not outer aspect, wide ends
acute chest syndrome
hematuria, papillary necrosis, ESrD
proliferative neuropathy
retinal infarcts
retinal detachment
priaprism
increased risk infection |
|
|
Term
|
Definition
| painful swelling of dorsa of hands and feet in infant or 4-6mo due to avascular necrosis of metacarpal and metatarsal bones in sickle cell |
|
|
Term
| acute chest syndrome sx, tx |
|
Definition
repetitive pulmonary infarcts, new infiltrate and alveolar consolidation
fever, CP, tachycardia, wheezing, cough, hypoxia
tx: transfusion UP TO 10 Hb, 3rd gen ceph, macrolide, anticoagulation, IVF, pain control |
|
|
Term
| common infections in sickle cell |
|
Definition
anaplastic crisis: parvoB19
functional asplenia increases risk: H. influenza, S. pneumo, N. meningitidis, klebsiella
osteomyelitis: salmonella |
|
|
Term
| treatment of retained priaprism in sickle cell |
|
Definition
hydralazine, nifedipine, stilbesterol (antiandrogen)
if >3h it is medical emergency |
|
|
Term
|
Definition
decreased haptoglobin
increased indirect bilirubin
decreased Hct
increased reticulocytes
increased HbF and HbS
decreased HbA
sickledex solubility test
x-ray: fish mouth vertebrae, lung infiltrates
blood smear: target cells, nucleated RBC, sickle cells |
|
|
Term
|
Definition
vaccinations
prophylactic antibiotics
folic acid due to chronic hemolysis
hydroxyuria increases HbF reducing crisis
blood transfusion last resort
marrow transplant |
|
|
Term
| how to treat sickle cell crisis |
|
Definition
PO/IVF
morphine
keep warm
O2 if hypoxia |
|
|
Term
| hereditary spherocytosis sx, tx |
|
Definition
AD defect in spectrin causing sphere RBC
splenomeagly, gall stones, hemolytic anemia, jaundice
positive osmotic fragility test
spherocytes
pseudohyperkalemia due to lysis
folic acid, RBC transfusion if severe anemia, splenomeagly |
|
|
Term
|
Definition
| XR causes H2O2 build up denaturing Hb percpipitating heinz bodies reducing RBC flexability and their sequestered by spleen |
|
|
Term
| G6PDH deficiency types and risk factors |
|
Definition
mild (AA): hemolytic episodes self limited, only involve old RBC
severe (mediterranean): involve young and old RBC, causes severe hemolytic anemia |
|
|
Term
|
Definition
| sulfonamides, nitrofuronatoin, primaquine, dimercaprol, fava beans, infection |
|
|
Term
|
Definition
bite cells: splenic macrophages take bites out
heinz bodies: abnormal Hb percpipitates in RBC
measurement of G6PDH (normal during episode because deficient RBC were just exposed of) |
|
|
Term
| paroxysmal nocturnal hemoglobinuria sx, dx, tx |
|
Definition
deficiency in proteins that protect from complement damage
chronic hemolysis, thrombosis/bud chiari, abdominal and back pain, pancytopenia, normochromic normocytic anemia
hams test: cells incubated in acid triggering complement allowing lysis
sugar water test: pt serum mixed in sucrose, hemolysis occurs
flow cytometry for CD55/CD59 most sensitive and specific
tx: prednisone |
|
|
Term
| hemophilia: platelet ct, bleeding time, PT, PTT |
|
Definition
platelet normal
bleeding normal
PT normal
PTT long |
|
|
Term
| vWD: platelet ct, bleeding time, PT, PTT |
|
Definition
platelet normal
bleeding long
PT normal
PTT long |
|
|
Term
| ITP: platelet ct, bleeding time, PT, PTT |
|
Definition
platelet low
bleeding long
PT normal
PTT normal |
|
|
Term
| TTP: platelet ct, bleeding time, PT, PTT |
|
Definition
platelet low
bleeding long
PT normal
PTT normal |
|
|
Term
| DIC: platelet ct, bleeding time, PT, PTT |
|
Definition
platelet low
bleeding long
PT long
PTT long |
|
|
Term
| Heparin: platelet ct, bleeding time, PT, PTT |
|
Definition
platelet normal
bleeding normal
PT normal
PTT long |
|
|
Term
| Warfarin: platelet ct, bleeding time, PT, PTT |
|
Definition
platelet normal
bleeding normal
PT longer (second)
PTT long (first) |
|
|
Term
| Aspirin: platelet ct, bleeding time, PT, PTT |
|
Definition
platelet normal
bleeding long
PT normal
PTT normal |
|
|
Term
| liver disease: platelet ct, bleeding time, PT, PTT |
|
Definition
platelet normal
bleeding normal
PT long
PTT long |
|
|
Term
|
Definition
|
|
Term
| based on platelet number what are the symptoms of thrombocytopenia |
|
Definition
>100,000 abnormal bleeding unusual
20,000-70,000 bleeding after surgery or trauma
<20,000 minor spontaneous bleeding
<5,000 major spontaneous bleeding |
|
|
Term
| idiopathic immune thrombocytopenia purpura cause, sx, tx |
|
Definition
IgG to platelets, removal in spleen
self limited in kids only
no neuro signs, fever, or renal failure
normal peripheral smear
tx: steroids, IVIG, plasmaphoresis, splenectomy often curative |
|
|
Term
| thrombotic thrombocytopenic purpuria cause, tx |
|
Definition
HUS with fever and altered mental status
caused by endothelial injury, E. coli O157:H7, malignancy, HELLP
deficient in ADAMTS12 (vWF cleaving metalloprotease) that degrades large multimeres, so big vWF allow for MORE clotting
plasmaphoresis ASAP, steroids |
|
|
Term
| heparin induced thrombocytopenia cause, sx, diagnosis |
|
Definition
Ab to heparin platelet factor 4 activates neighboring platelets causing clotting and consumption
hypercoaguability and thrombocytopenia
sensitive: serotonin release assay
specific: heparin induced platelet aggregation assay |
|
|
Term
| bernard-soulier signs, cause |
|
Definition
AR subendothelial deficiency in platelet GP1B-IX preventing adhesion
large platelets on smear
LOW RISTOCETIN ASSAY
mild platelet count decrease |
|
|
Term
| glansmanns thromboblasthenia cause, sx |
|
Definition
AR deficiency in GPIIb/IIIa, decreased aggregation
prolonged bleeding time
platelet count normal |
|
|
Term
| what is vWF, how does it work |
|
Definition
factor VIII has two parts...
coagulation protein: made in liver, functions in clot formation
antigenic protein (vWF): made in enothelial cells and megakaryocytes, functions in platelet adhesion via G1b/IX to subendothelium and binds factor VIII coagulant protein and protects it from degradation |
|
|
Term
|
Definition
HIGH RISTOCETIN COFACTOR ASSAY
easy bruising, excessive bleeding, gingival bleeding, , GI bleeding |
|
|
Term
|
Definition
desmopressin DOC: ADH analog increases vWF secretion and circulating VIII (not effective in type III where vWF is absent)
factor VIII: given at surgery
cryoprecipitate: for refractory bleeding
OC: for menorrhagia
avoid NSAIDS |
|
|
Term
|
Definition
XR factor VIII deficiency (coagulation protein)
bleeding, hemarthrosis, hemorrhage after surgery, intracranial bleeds, intramuscular hematoma, hematuria, GI bleeding, hemospermia, retroperitoneal hematoma |
|
|
Term
|
Definition
clotting factor replacement
DDAVP helps with mild disease because it increases VIII
hemoarthrosis: analgesia, immobilization, ice, non-weight bearing, synovectomy |
|
|
Term
| cause of hemophilia B, C, D |
|
Definition
B: factor IX deficiency
C: factor XI deficiency (Jews)
D: factor XII deficiency |
|
|
Term
|
Definition
| infection, usually gram negative |
|
|
Term
|
Definition
treat cause, O2, IVF, FFP, maintain BP, platelets, cryopercipitate
heparin inhibits clotting and prevents consumption of clotting factors |
|
|
Term
|
Definition
AD lack of AT3 inhibitor of thrombin increasing thrombosis
anticoagulate with anything but heparin |
|
|
Term
| antiphospholipid antibody syndrome cause, risks, signs |
|
Definition
Ab to phospholipids causes hypercoaguability
risks: pregnancy, lupus, autoimmune
signs: recurrent fetal loss, thrombocytopenia, thrombosis |
|
|
Term
| protein C and S deficiency cause |
|
Definition
protein C deficiency is AD, it cannot inhibit factors V/VIII allowing unregulated fibrin synthesis
protein S is a cofactor for protein S, protein S deficiency causes protein C deficiecy |
|
|
Term
|
Definition
| mutation in factor V gene, protein c cant inactivate factor V leading to unregulated prothrombin activation |
|
|
Term
| monoclonal gammopathy of undetermine significance |
|
Definition
>75yo, premalignant clonal plasma cell proliferation, asymptomatic, <10% plasma cells in marrow, bence jones <1g/24h, IgG spike <3g
<20% develop multiple myeloma in 10-15y but almost all multiple myeloma patients had MGUS |
|
|
Term
| signs of multiple myeloma |
|
Definition
hyperviscosity: retinopathy, CVA
osteolytic lesions: bone pain, fracture, vertebral collapse
anemia: weak, fatigue, weight loss, pallor
renal failure: myeloma nephrosis causes bence jones casts, hypercalcemia
recurrent infections: Ig not functional
cardiac amyloidosis: restrictive cardiomyopathy
bence jones protein in urine (light chains), IgM spike in serum (heavy chains)
lytic bone lesions
RBC in rouleadux formation
hypercalcemia
decreased anion gap (Ig are positive) |
|
|
Term
| treatment multiple myeloma |
|
Definition
autologous hematopoietic cell transplant: if young and asymptomatic
systemic chemo: alkylating agents
radiation: if refractory
bisphosphonates |
|
|
Term
| waldenstorms macroglobulinemia cause, sx, tx |
|
Definition
malignant plasmacytoid lymphocytes that make IgM paraprotein which causes hyperviscosity
fatigue, weight loss, neuro signs, lymphadenopathy, splenomeagly, anemia, bleeding, hyperviscosity (retinal vessel dilation, blindness)
IgM >5g/dL, bence jones proteinuria, NO BONE LESIONS
tx: chemo, plasmaphoresis |
|
|
Term
| neutropenic fever signs, tx |
|
Definition
cause: marrow failure (toxin, drug), marrow invasion (malignancy), hypersplenism, SLE, AIDS
signs: fever >1h, neutrophils <500, septicemia, cellulitis, pneumonia, NEVER DO PROSTATE EXAM BLEEDING RISK
tx: isolation, masks, handwashing, positive pressure, if low risk give cipro and amoxicillin, if high risk give cefepime, penicillin, ceftazidine, or carbapenem |
|
|
Term
| explain the 4 types of hypersensitivity |
|
Definition
1) antigen reacts with IgE causing histamine release
2) autoantibodies to cell antigens
3) autoantibodies cause immune complexes that deposit in tissues
4) T cells present antigens to macrophages and secrete lymphoines that induce macrophages to destory surrounding tissue |
|
|
Term
|
Definition
nodular sclerosis: M=F, 30yo, bands of collagen envelope pools of reed sternburg cells
mixed cellularity: many reed sternburg on pleomorphic background
lymphocyte predominate: few reed sternberg, many B cells
lymphocyte depletion, worse prognosis, mix of reactive cells, 60yo |
|
|
Term
|
Definition
1) confined to single node
2) involves 2+ nodes but confined to same side of diaphragm
3) involves both sides of diaphragm
4) dissemination extra lymphatic |
|
|
Term
|
Definition
fever, weight loss, night sweats, puritis, cough if mediastinal, HSM, painless lymphadenopathy firm rubbery (cervical, axillary, mediastinal), symptoms worsen with alcohol
REED STERNBURG CELLS (2 OWL EYE NUCLEI, B CELL)
inflammatory infiltrate inreaction to reed sternburg cells |
|
|
Term
|
Definition
| 10-30yo, >50yo, EBV, male |
|
|
Term
| when should you biopsy a node |
|
Definition
| >1cm for <4wk not due to infection |
|
|
Term
| general treatment of non-hodgkins |
|
Definition
CHOP: cyclophosphamide, hydroxydanomycin/doxyrubicin, oncovin/vincristin, prednisone
rituximab: monoclonal antibody against CD20 |
|
|
Term
| intolent, intermediate and high grade lymphomas, which is most common |
|
Definition
indolent: small lymphocytic, follicular cleaved cell
intermediate: diffuse large cell (most common)
high: lymphoblastic, burkitts (small non-cleaved) |
|
|
Term
| small cell lymphoma signs |
|
Definition
| same as CLL just in node, can become CLL |
|
|
Term
| follicular cleaved cell lymphoma |
|
Definition
t(14;18)
painless, peripheral lymphadenopathy
cleaved cells (dented lymphocytes)
can transform to large cell
locally radiation sensitive |
|
|
Term
| diffuse large cell lymphoma |
|
Definition
B cell origin, associated with HIV
locally invasive extranodal mass
85% cure with CHOP if not HIV associated |
|
|
Term
|
Definition
T cell origin, can progress to T-ALL
seen in kids, responds to combo chemo |
|
|
Term
|
Definition
t(8;14), B cell origin
risk EBV, african, HIV
starry sky pattern (dark lymphocytes, light macrophages, macrophages engulfing apoptotic neoplastic cells)
agressive but responds to chemo |
|
|
Term
|
Definition
Th cell lymphoma of skin
pink, puritic, scaly plaque, tumor later (elevated)
AA>caucasian
disseminates to nodes, blood, organs
cribiform shaped lymphocytes
radiation and topical chemo |
|
|
Term
|
Definition
| mycosis fungoides that has progressed from the skin into the blood stream |
|
|
Term
|
Definition
NO BONE PAIN, fatigue, easy bruising, pallor, infections, thrombocytopenia, HSM, skin nodules
AUER RODS: granules of eosinophilic rods
MYELOPEROXIDASE POSITIVE WITH SUDAN BLACK |
|
|
Term
|
Definition
all-trans retinoic acid (ATRA) ASAP and chemo
marrow transplant gives best chance at survival |
|
|
Term
|
Definition
B cell proliferation, in adults philaldephia t(9;22) bcr-abl
risk: downs, neurofibromatosis, 2-5yo, white
anemia, fatigue, weakness, pallor, infection, thrombocytopenia, HSM, lymphadenopathy, BONE PAIN due to periosteal invasion, meningitis, seizure, UNILATERAL PAINLESS TESTICULAR ENLARGEMENT, ANTERIOR MEDIASTINAL MASS
PAS AND TdT POSITIVE BLAST CELLS |
|
|
Term
|
Definition
| combination chemo, marrow transplant best change of survival, worst prognosis if CnS involvement (B cell phenotype, increased LDH, rapid leukemic cell proliferation) |
|
|
Term
|
Definition
B cell proliferation, mature, non-functional
>60yo, white, male, western
painless lymphadenopathy, respiratory and skin infections, fatigue, HSM, fevers, night sweats, pallor, bruising, SEVERE HEMOLYTIC ANEMIA
C5 IS HIGHLY SENSITIVE
WBC 50,000-200,000
SMUDGE CELLS: BROKE WHEN ON SLIDE |
|
|
Term
|
Definition
philadelphia t(9;22) bcr-abl activates TK
>40yo, radiation exposure
chronic: fever, night sweats, weight loss, fatigue, infections, bruising, anemia, HSM, lymphadenopathy
BLAST CRISIS: worse symptoms
DECREASED LEUKOCYTE ALKALINE PHOSPHATASE ACTIVITY |
|
|
Term
|
Definition
imatinib: TK inhibitor targets dysfunctional bcr-abl
blast crisis: add stem cell transplantation
young patient: marrow transplant |
|
|
Term
|
Definition
B cell proliferation, middle aged male
fatigue, infection, ABDOMINAL FULLNESS, SPLENOMEAGLY, DOES NOT HAVE NIGHT SWEATS OR FEVER OR LYMPHADENOPATHY
mononuclear, reticular chromatin, abundent cytoplasm FRIED EGG APPEARANCE, HAIRY PROJECTIONS |
|
|
Term
| tumor lysis syndrome cause |
|
Definition
| hyperuricemia due to high rate of release of intracellular contents |
|
|
Term
|
Definition
malignant clonal proliferation of hematopoetic stem cells leading to excessive RBC, WBC, and platelets INDEPENDENT of EPO
associated with JAK2 TK activity |
|
|
Term
|
Definition
headache, dizziness, tinnitus, vertigo, HSM, blurred vision, amaurosis fugax, scintillating scotoma, opthalmic migraine, DVT, PE, CVA, MI, claudication, budd-chiari, superifical thrombophelbitis, erythromeaglia (burning pain in hands and feet, erythema, pallor, cyanosis), puritis esp after warm bath, facial plethora, hyperuricemia
EPO REDUCED, HYPERCELLULAR MARROW |
|
|
Term
| treatment polycythemia vera |
|
Definition
| phlebotomy, hydroxyuria if at risk of thrombosis (<70yo, platelet <1,500,000, CV risk), INFa if refractory erythrocytosis or puritis, aspirin, allopurinol |
|
|
Term
| signs of myelodysplastic syndromes |
|
Definition
pseudo-pelger-huet anomly: nuclei have two lobes, connected by thin strand
hyperlobulated nuclei in neutrophils
nucleated RBC with howell jolly bodies, macrocytic anemia
platelets large and agranular |
|
|
Term
| essential thrombocythemia cause, sx, dx, tx |
|
Definition
diagnosis of exclusion after reactive thrombocytosis and other myeloproliferative disorders ruled out
CVA, bleeding, splenomeagly, pseudohyperkalemia, erythromelagia
platelets >600, peripheral smear with hypogranular platelets, marrow biopsy with megakaryocytes
tx antiplatelet antigens (anagrelide), low dose aspirin, hydroxyuria if severe thrombosis |
|
|
Term
|
Definition
prophobilinogen deaminase deficiency leads to increased aminolevulinic acid and prophobilinigen
percipitants: stress, smoking, drugs, female
ABDOMINAL PAIN, vomiting, constipation, tachycardia, HTN, muscle weakness, extremity pain, psych symptoms, RED URINE, NO RASH
AMINOVOLEVILINIC ACID AND PORPHOBILINIGEN IN URINE |
|
|
Term
|
Definition
RNA retrovirus attaches to CD4 surface (macrophages, dendritic cells, Th) enters and uncoats, RNA is transcribed into RNA by reverse transcriptase, CD4 depletion weakens immune system
HIV 1 progresses faster, HIV2 is less infectious early but more infectious later, less common in US, and more resistant to treatment |
|
|
Term
| risk of HIV transmission from a needle stick |
|
Definition
|
|
Term
| risk of HIV from semen or vaginal fluid |
|
Definition
1/1000 semen
1/3000 vaginal |
|
|
Term
|
Definition
| acute retroviral syndrome: 2-4wk post exposure, last 2wk, fever, lethargy, myalgia, sore throat, truncal maculopapular rash, headache, mucosal ulcers |
|
|
Term
|
Definition
| CD4 <200, opportunistic infections and malignancies, weight loss, night sweats, neurological changes, pulmonary, GI, cutaneous and systemic infections |
|
|
Term
|
Definition
PCR RNA viral load: seen before Ab response, can assess antiretroviral therapy
CD4 count: best indication of immune system status
p24 assay: sensitive alternative to viral load
ELISA: detects Ab positive in 1-12wk, negative excludes HIV, sensitive
western blot: specific, done after 2 positive ELISA, conformatory |
|
|
Term
| treatment of occupational HIV exposure |
|
Definition
tenofovir + emtricitabine + raltegavir
HIV Ab test immediatly, at 6wk, 3mo, 6mo
continue treatment for 4wk |
|
|
Term
|
Definition
2 NRTI + 1(NNRTI or PI) often adding ritonavit to increase PI activity
monitor viral load q3-6mo, goal to reduce to <50 |
|
|
Term
| treatment of HIV in pregnancy |
|
Definition
transmission is rare when viral load <1000
zidovudine during labor
zidobudine to newborn for 6wk, test viral load at birth then at 6mo (Ab will always be positive from mom)
never breast feed |
|
|
Term
|
Definition
PCP: TMP-SMX when CD4 <200
toxo: TMP-SMX when CD4 <100
MAC: azithromycin when CD4 <100
TB: PPD yearly
pneumovax: q5-6y
influenza: yearly |
|
|
Term
| AIDS dermatologic inections: CD4, types, treatment |
|
Definition
HSV (shingles), mulloscum, syphilis
acyclovir, valacyclovir, famicyclovir, foscarnet if resistant |
|
|
Term
| kaposki sarcoma cause, sx, dx, tx |
|
Definition
HHV8, vascular tumor of lymphatic endothelium
AIDS CD4 <500 (usually <250)
painless raised brown black or purble papules on face, chest, genitals, outh, organs
dx biopsy
HAART, alretinoin, chemo, laser, radiation |
|
|
Term
| AIDS oral lesions: CD4, types |
|
Definition
<500
thrush, HSV, CMV, HBV (hairy leukoplakia), kaposkis |
|
|
Term
| AIDS diarrhea: CD4, cause, treatment |
|
Definition
<500
prolonged diarrhea, malaise, weight loss, abdominal pain
strongloides: ivermectin
isospora: TMP-SMX
cryotpsporidium: nitazoxonide
others: E. coli, shigella, salmonella, camplobacter, CMV, giardia, MAC, antibiotics |
|
|
Term
| AIDS wasting syndrome: CD4, signs, tx |
|
Definition
<300 (usually <100)
loss of <10% body weight, chronic diarrhea (2+/d) or fever and persistent weakness
EMG with peripheral nerve dysfunction
exercise, steroids, HAART, nutrition, megestrol appetite stimulant, testosterone if hypogonadism |
|
|
Term
| AIDS cyccidioidomycosis: CD4, sx, tx |
|
Definition
<250
BL reticular infiltrates on CXR, positive Ab, cough, fever, dyspnea
fluconazole, intraconazole, amphotericin B |
|
|
Term
| AIDS dementia: CD4, sx, tx |
|
Definition
<200 (early stages can be >200)
early subtle imairement of recent memory, intellectual decline, apathy
later confusion, motor change, mental status change, tremor, aphasia
cerebral atrophy
ELEVATED B2 MICROGLOBULIN IN CSF
HAART that crosses BBB |
|
|
Term
| AIDS pneumonia: CD4, causes, signs, tx |
|
Definition
S. pneumo, H. influena, nocardia
rapid onset, productive cough, high fever, hypoxia, present 2+/y
lobular consolidation or diffuse infiltrates
cephalosporins, lactams, macrolides |
|
|
Term
| what is the leading COD in AIDS |
|
Definition
|
|
Term
| AIDS esophagitis: CD4, signs, dx, tx |
|
Definition
candidia, HSV, CMV
odontophagia, dysphagia
endoscopy and biopsy, gram stain of scrapings
topical or oral fluconazole, nystatin swish and swallow, amphotericin if resistant |
|
|
Term
| what cancer is indicitive of AIDS, tx |
|
Definition
cervical cancer, pap and biopsy dx, tx topical 5-FU and resection, radiation, chemo
primary CNS lymphoma: headache, confusion, focal neuro signs, requires chemo and radiation
non-hodgkins lymphomas |
|
|
Term
| pneumocystis jurovecci: CD4, signs, dx, tx |
|
Definition
<200
gradual onset, dry cough, dyspnea on exertion, fever, BL CENTRAL INFILTRATES, INCREASED LDH
TMP-SMX, steroids if severe |
|
|
Term
| histoplasmosis: CD4, signs, tx |
|
Definition
<150
abdominal pain, GI bleeding, skin lesions, dyspnea, meningitis, fever, weight loss, fatigue, adrenal insufficiency, BL infiltrates on CXR, positive antigen in urine or serum
long term amphotericin B or intrconazole |
|
|
Term
| toxoplasmosis: CX4, signs, dx, tx |
|
Definition
<100
headache, confusion, focal neuro signs, fever, change in mental status, seizure, positiveIgG, ring enhancing lesions on CT/MRI in basal ganglia and white matter
TMP-SMX, leukovorin or clindamycin |
|
|
Term
| progressive multifocal leukoencephalopathy: CD4, cause, signs, dx, tx |
|
Definition
<100
JC virus, demyelinating
motor defects, mental status change
positive PCR of CSF for JC DNA
HAART |
|
|
Term
| cryptococcus: CD4, signs, dx, tx |
|
Definition
<50
meningitis, brain abscess, headache, fever, irritability, dizzy, confusion, seizure, stiff neck, mental status change
elevated LP pressure, budding round yeast, india ink positive, antigen positive in urine or serum
tx amphotericin B and flucosine for 14d then fluconazole for 10wk |
|
|
Term
|
Definition
<50
retinitis, UL vision loss, colitis, diarrhea, esophagitis
viral titer, yellow infiltrates with hemorrhage on fundoscopic exam
ganicyclovir, foscarnet, valganciclovir |
|
|
Term
|
Definition
<50
fatigue, diarrhea, abdominal pain, HSM, wasting, lymphadenopathy, anemia
blood or stool culture
clathromycin + ethembutol + rifampin |
|
|
Term
|
Definition
falciprum: constant fever, worst, most common
ovale: fever q48h, can get in liver
vivax: fever q48h, can get in liver
malariae: fever q72h |
|
|
Term
|
Definition
onset weeks to months later, headache, myalgia, fevers, chills, diaphresis, abdominal pain, nausea, vomiting, diarrhea, pulmonary edema, renal insufficiency, LOC
HIGH LDH, ATYPICAL LYMPHOCYTES, THROMBOCYTOPENIA, GEMISA STAIN RING SHAPE IN RBC |
|
|
Term
|
Definition
chloroquine: DOC unles resistance
quinidine + tetraclcine: if resistant
atovaquone-proquanil + mefloquine: alt
quinidine + doxy: for falciprum
primaquine: add for ovale and vivax |
|
|
Term
|
Definition
mefloquine: if chlorquine resistant (falciprum: africa, carribean)
chlorquine: if not resistant |
|
|
Term
| ehrilchosis cause, sx, tx |
|
Definition
intracellular bacteria, transmitted by tick, reservior on deer
chills, malaise, fever, rash, renal failure, GI bleeding
tx tetracycline or doxy 1wk |
|
|
Term
|
Definition
infection of B cells leading to abnormal T cells (downey cels) that fight B cells
transmitted by saliva
incubation 2-5wk |
|
|
Term
|
Definition
fever 104, painful lymphadenopathy, erythematous EXUDATIVE PHARYNGITIS, malaise, myalgia, fatigue, SPLENOMEAGLY, MACULOPAPULAR RASH IF GIVEN AMPICILLIN, eyelid edema
HETEROPHILE AB, EBV ANTIBODY TESTING, LYMPHOCYTIC LEUKOCYTOSIS, ATYPICAL LYMPHOCYTES, elevated LFT, hemolytic anemia, thrombocytopenia |
|
|
Term
|
Definition
| seen in sexually active young adults with fever, chills, fatigue, headache, splenomeagly, not lymphadenopathy or heterophile antibodies |
|
|
Term
| fanconi anemia cause, dx, tx |
|
Definition
fatigue, dyspnea, infections, short stature, cafe-au-lait spots, hypo/hyperpigmentation, horseshoe kidney, absent or hypoplastic thumbs
decreased Hct.Hb, platelets, WBC, increased AFP, marrow with hypocellularity
tx antibiotics, transfusions, marrow or stem cell transplants |
|
|
Term
| diamond black fan anemia cause, dx, tx |
|
Definition
congenital pure RBC anemia, defect in progenitor cells
fatigue dyspnea, cyanosis, pallor, craniofascial abnormalities, murmurs, MR, hypogonadism, thumb abnormalities
decreased Hb/Hct and reticulocytes, increased EPO |
|
|
Term
| neuroblastoma cause and associations |
|
Definition
tumor of neural crest cells that arises in adrenal glands or SNS ganglia
assoc: neurofibromatosis, tuberous sclerosis, pheochromocytoma, blackwith-weidmann, turner syndrome, low maternal folate |
|
|
Term
|
Definition
abdominal distension and pain,w eight loss, maialse, bone pain, diarrhea, abdomial mass, HTN sustained, horners, propotosis, movement disorders, hepatomeagly, fever, periorbital bruising
increased VANILLYMANDELIC AND HOMOVANILLIC ACID IN URINE, CT with localized adrenal or ganglion tumor
surgical resection, chemo, radiation |
|
|
Term
| rhabdomyosarcoma cause, sx, dx, tx |
|
Definition
tumor of striated muscle in kids
painful soft tissue mass, swelling, large tumor causes mass effect
biopsy is diagnosic
surgical debulking, radiation, chemo |
|
|
Term
| langerhans cell histocytosis cause risk, diagnosis |
|
Definition
proliferatioon of langerhans cells (histocyte infiltration)
assoc with interstitial lung disease and smoking
diagnosis CD1a specific |
|
|
Term
| langerhans histocytosis types |
|
Definition
1) eosinophilic granuloma: only involves bone
2) hand schuller christian; young kid, cystic defect of skull, DI, exopthalmos
3) letter siwe: systemic <2yo, puritic rash, bone lesions, enlarged nodes, HSM
4) pulmonary: smoker, 20-40yo, dyspnea, non-productive cough, fatigue, weight loss, CXR with BL nodules, cysts, honeycombs, lytic lesions on ribs, TX steroids |
|
|
Term
|
Definition
| <10yo, pancytopenia (petechiae, brusing, pallor, fatigue, infection), thumb abnormalities, cafe au lait spots, renal abnormalities, microcephaly, short stature |
|
|
Term
| diagnosis and cause of fanconi anemia |
|
Definition
chromosome breakage test
DNA repair defect |
|
|
Term
| complications of fanconi anemia |
|
Definition
| AML, myelodysplastic syndrome, liver tumors |
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