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IM - Hematology
Nova Orlando - Rotations
120
Health Care
Graduate
09/25/2011

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Term
at what Hb level is transfusion recommended?
Definition
< 7 g/dL
Term
1 unit of packed RBC's increases Hb and Hct by how much?
Definition
Hb by 1 point
Hct by 3 points
Term
what is in PRBC vs FFP vs cryoprecipitate
Definition
PRBC - no platelets or clotting factors - mix with NS to infuse faster.
FFP - all clotting factors, no RBC/WBC/platelets
cryoprecipitate - factor 8 and fibrinogen. used for hemophilia A, DIC, and vWD.
Term
1 unit of platelets raises platelet count by how much?
Definition
10,000
Term
what is the importance of retic count in anemia?
Definition
tell whether or not the body is able to respond adequately to the anemia.
Retic count > 2% = adequate bone marrow response.
Retic count < 2% = inadequate RBC production by the bone marrow
Term
types of microcytic anemia (3)
Definition
iron deficiency (MC)
thalassemia
ring sideroblastic anemias (lead poisoning, pyridoxine deficiency, toxic effects of alcohol)
Term
casues of macrocytic anemia (3)
Definition
nuclear defect - B12, folate deficiency
liver disease
stimulated erythropoiesis
Term
causes of normocytic anemia (5)
Definition
aplastic
bone marrow fibrosis
tumor
anemia of chronic disease
renal failure
Term
MCC of anemia worldwide
Definition
iron deficiency
Term
lab results in iron deficiency anemia
Definition
decreased Fe
increaed TIBC
elevated transferrin levels
decreased serum iron
microcytic hypochromic RBC's on smear
Term
gold standard for dx of iron deficiency anemia
Definition
bone marrow biopsy (rarely performed)
Term
methods of iron replacement
Definition
oral = ferrous sulfate
IV/IM = iron dextran
Term
who is beta thalassemia most common in?
Definition
people of Mediterranean, Middle Eastern, and Indian ancestry.
Term
effects of alpha thalassemia
Definition
alpha chains are a component of all types of hemoglobin, so alpha thalassemia affects alpha and beta chains.
Term
pts with transfusion dependent thalassemia can develop what?
Definition
iron orverload that leads to CHF - these pts are often treated with desferrixoamine (chelating agent)
Term
how do you diagnose beta-thalassemia
Definition
hemoglobin electrophoresis - increased hemoblogin F
peripheral blood smear - microcytic hypochromic anemia
Term
tx of beta thalassemia major (Cooley's anemia).
Definition
frequent PRBC transfusion
Term
dx of b-thalassemia minor
Definition
heterozygous b-chain thalassemia
Term
types of alpha thalassemia
Definition
silent carriers - mutation/deletion of only one a locus
a-thalassemia trait - mutation/deletion of two alpha loci
Hg H disease - mutation at 3 alpha loci
tx = frequent PRBC transfusion, splenectomy can be helpful
mutation at all 4 loci is either fatal at or shortly after birth
Term
clinical findings of siderblastic anemia
Definition
increased serum iron and ferritin
normal TIBC
ringed sideroblasts in bone marrow
Term
causes of anemia of chronic disease (4)
Definition
chronic infection - TB, lung abscess
cancer - lung, breast, Hodgkin's
inflammation - RA, SLE
trauma
release of cytokines has a suppressive effect on erythropoeisis
Term
low serum iron
low TIBC
low serum transferrin levels
increased serum ferritin levels
Definition
anemia of chronic disease
Term
causes of aplastic anemia (5)
Definition
idiopathic (majority)
radiation exposure
meds - chloramphenicol, sulfonamides, gold, carbamazepine
viral infection - parvovirus, hep C, Hep B, EBV, CMV, zoster, HIV
chemicals - benzene, insectacides
Term
tx of aplastic anemia
Definition
bone marrow transplantation
transfusion of PRBC and platelets (use judiciously)
immunosuppression
Term
how mcuh B12 do we store in the body?
Definition
B12 stores in the liver can last a person for 3 or more years
Term
main dietary sources of B12
Definition
meat and fish
Term
how/where is B12 absorbed?
Definition
bound to intrinsic factor (produced by gastric parietal cells),so it can be absorbed by the terminal ileum
Term
MCC of B12 deficiency anemia
Definition
impaired absorption
Term
how does pernicious anemia differ from B12 deficiency anemia
Definition
pernicious anemia is an autoimmune disorder resulting in inadequate production of intrinsic factor and thus impaired absorption of B12
Term
lab findings in b12 deficiency anemia
Definition
megaloblastic anemia
hypersegmented neutorphils
B12 levels < 100 pg/mL
serum methylmalonic acid and homocysteine levels are elevated
Term
diagnostic test for B12 deficiency
Definition
Schilling Test
Term
how much folic acid does the body store?
Definition
limited - inadequate intake over 3 months can lead to deficiency
Term
main source of folate
Definition
green vegetables - overcooking them can remove the folate
Term
Heinz bodies
Definition
G6PD anemia
Term
schistocytes
Definition
suggest intravascular hemolysis ("trauma" or mechanical hemolysis)
Term
spherocytes of helmet cells suggest
Definition
extravascular hemolysis
Term
pathogenesis of sickle cell diesase
Definition
hemoglobin S replaces hemoglobin A
Term
aplastic crisis in sickle cell anemia can be precipitated by
Definition
viral infections such as human parvovirus B19.
tx = blood transfusion
Term
clinical findngs in sickle cell anemia secondary to vaso-occlusion
Definition
painful crisis involving the bone
hand-foot syndrome - painful swelling of dorsa ofhands and feet seen in infancy and childhood caused by AVN of the metacarpal and metatarsal bones.
acute chest syndrome - due to repeated episodes of pulmonary infarctions
repeated splenic infarctions
AVN in joints - MC = hip
priapism
CVA
ophthalmologic complications
chronic leg ulcers
abdominal crisis
Term
splenic sequestration syndrome
Definition
sudden pooling of blood into the spleen results in rapid development of splenomegaly and hypovolemic shock - potentially fatal complication of sickle cell disease and B-thalassemia. MC in children because they have intact spleens.
Term
sickle cell patients should be vaccinated against what?
Definition
pneumoniae, H influenzae, N. meningitidis
Term
prophylactic tx of kids with sickle cell disease
Definition
PCN from age 4m-6yrs
Term
tx of sickle cell crisis
Definition
hydration
pain mangement with morpine
keep pt warm
supplemental O2
hydroxyurea - enhances Hb F
Term
lab findings in hereditary spherocytosis
Definition
RBC osmotic fragility
elevated retic count and elevated MCHC
blood smear = sphere shaped RBCs
direct coombs test is negative
Term
tx of choice for hereditary spherocytosis
Definition
splenectomy
Term
known precipitants of G6PD deficiency
Definition
sulfonamides, nitrofurantoin, primaquine(antimalarial), dimercaprol, fava beans, infection
Term
clinical features of G6PD
Definition
more severe in people of Mediterranean descent
episodic hemolytic anemia that is usually drug induced
dark urne and jaundice
Term
dx of G6PD
Definition
blood smear = bite cells and heinz bodies
deficient NADPH formation on G6PD assay
low levels of G6PD (althogh may be normal during hemolytic episode)
Term
autoimmune hemolytic anemia (AIHA)
Definition
production of autoantibodies toward RBC membrane antigen which leads to destruction of these RBCs
Term
warm vs cold AIHA
Definition
warm - IgG binds optimally at warm temps causes extravascular hemolysis. causes = lymphoma, leukemia, collagen vascular disease, drugs
cold - IgM binds well at cold temps causes intravascular hemolysis. causes = idiopathic or
Term
warm vs cold AIHA
Definition
warm - IgG binds optimally at warm temps causes extravascular hemolysis. causes = lymphoma, leukemia, collagen vascular disease, drugs
cold - IgM binds well at cold temps causes intravascular hemolysis. causes = idiopathic or infection.
Term
dx of AIHA
Definition
direct coombs test
RBC's coated with IgG = positive = warm AIHA
RBC's coated with complement alone - dx is cold AIHA
Term
tx of AIHA
Definition
often none is necessary.
warm - glucocorticoids, splenectomy, immunosuppression, RBC transfusion, folic acid supplement.
cold - avoid exposure to cold, RBC transfusion, chemotherapeutic agents, steroids are not beneficial!
Term
definition of thrombocytopenia
Definition
platelet count < 150,000
Term
types of heparin induced thrombocytopenia
Definition
HIT1 - heparin directly causes platelet aggregation, seen < 48 hrs after initiating
HIT2 - heparin induced antibody mediated injury to platelet - seen 3-12 days after initating heparin. heparin should be d/c immediately
Term
clinical features of thrombocytopenia
Definition
cutaneous bleeding
mucosal bleeding
excessive bleeding after procedures or surgery
intracranial hemorrhage and heavy GI bleeding
Term
MCC of acute ITP
Definition
seen in kids and preceded by a viral infection.
usually self limited - 80% resolve spontaneously
Term
MCC of chronic ITP
Definition
usually seen in adult women age 20-40, spontaneous remissions are rare.
Term
diagnosis of ITP
Definition
platelet count usually < 20,000
bone marrow aspiration = increased megakaryocytes
increased amount of platelet associated IgG
Term
tx of ITP
Definition
adrenal corticosteroids
IV immune globulin
splenectomy
platelet transfusions
Term
hwo does TTP affect PT and PTT?
Definition
unaffected because there is no consumption of clotting factors
Term
tx of TTP
Definition
plasmapheresis (large volume)
corticosteroids and splenectomy
platelet transfusions are contraindicated!
Term
clinical findings of TTP
Definition
fever, altered mental status, HUS (microangiopathic hemolytic anemia, thrombocytopenia, renal failure)
Term
clinical findings of TTP
Definition
fever, altered mental status, HUS (microangiopathic hemolytic anemia, thrombocytopenia, r
Term
abnormally large platelets on peripheral smear think...
Definition
Bernard-Soulier Syndrome
Term
MC inherited bleeding disorder
Definition
von willebrand disease
Term
3 subtypes of vWF disease
Definition
1 = decreased
2 = abnormal
3 = absent
Term
lab findings with vWF disease
Definition
prolonged bleeding time - normal PT, normal or prolonged PTT
Term
tx of von willebrands disease
Definition
DDAVP - desmopressin (not effective in type 3)
factor 8 concentrates
avoid aspirin, NSAIDs, and cryoprecipitate
Term
characteristic type of bleeding for hemophilia
Definition
hemarthrosis, intracranial bleeding, intramuscular hematomas, retroperitoneal hematomas, hematuria or hemospermia
Term
lab findings in hemophilia A
Definition
prolonged PTT
Term
tx of hemophilia A
Definition
acute hemarthrosis - codeine with or without acetaminophen - avoid ASA/NSAID
clotting factor replacement - factor 8 concentrate
DDAVP
Term
tx of hemophilia B
Definition
limited to factor 9 concentrates
Term
what do PT, PTT, thrombin time, and bleednig time reflect?
Definition
PT = extrinsic pathway (warfarin)
PTT = intrinsic pathway (heparin)
TT = fibrinogen concentration
BT = platelet function
Term
normal PT, PTT, and bleeding time
Definition
PT = 11-15 seconds
PTT = 25-40 seconds
BT = 2-7 minutes
Term
causes of DIC (6)
Definition
infectin
obstetric complications
major tissue injury
malignancy
shock
snake venom (rattlesnakes)
Term
differentiate vitamin K deficiency and liver disease
Definition
liver disease - PT and PTT are elevated. TT, fibrinogen, and platelets are usually normal
vitamin K deficiency - Pt is prolonged, PTT, TT, platelet count, fibrinogen level S are normal
Term
dx of DIC
Definition
increased PT, PTT, BT, TT, fibrin split products, D-dimer
decreased - fibrinogen level, platelet count
peripheral smear reveals schistocytes
Term
vitamin K dependent clotting factors
Definition
2, 7, 9, 10, protein C and S
Term
pts with antithrombin 3 deficinecy don't respond to what medication?
Definition
heparin
Term
protein c deficiency leads to
Definition
increase in facotrs 5 and 8, which leads to unregulated fibrin synthesis
Term
factor 5 leiden deficinecy leads to
Definition
unregulated prothrombin activation and thus an increase in thrombotic events
Term
inherited hypercoagulable states (7)
Definition
antithrombin 3 deficiency
antiphospholipid antibody syndrome
Protein C deficiency
Protein S deficiency
Factor 5 Leiden
prothrombin gene mutation
hyperhomocystinemia
Term
heparin affects
Definition
PTT by inhibiting factors 2a and 10a.
half life of standard heparin is 1 hr
half life of LMWH is 3-24 hrs
Term
contraindications to heparin
Definition
previous HIT
active bleeding, GI/intracranial bleed
hemophilia, thrombocytopenia
severe HTN
recent surgery on eyes, spine, or brain
Term
reversal of heparin
Definition
half life is short, will cease to have effect 4 hrs after discontinuation
reverse effects with protamine sulfate.
administer FFP if severe bleeding occurs.
Term
warfarin affects
Definition
PT by inhibiting vitamin K dependent clotting factors
Term
administration of warfarin
Definition
start heparin first - once PTT is therapeutic being warfarin
continue heparin for atleast 4 days, once INR is therapeutic stop heparin and continue warfarin.
goal INR = 2-3
Term
reversing the effects of warfarin
Definition
vitamin K - can correct abnormal PT wihtin 4-10 hours. can make it difficult to return to the pt to therapeutic INR levels.
if significant bleed give FFP
Term
lab findings in MM
Definition
low hemoglobin
high calcium
high serum protein
poor renal function
lytic lesions in the low back, chest (ribs), or jaw.
Term
MCC of death in MM
Definition
recurrent infections
Term
dx of MM
Definition
serum and urine protein electrophoresis - monoclonal spike of IgG
atleast 10% abnormal plasma cells
lytic bone lesions
hypercalcemia
high serum protein
peripheral smear - rouleaux formation
elevated ESR
urine - bence jones proteins
leukopenia, thrombocytopenia, anemia
Term
tx of MM
Definition
systemic chemo
radiation therapy if no response to chemo and if disabling pain is present
transplantation - autologous peripheral blood stem cell transplantation
Term
how is Hodgkin's disease staged?
Definition
Ann Arbor staging system -
1 = single lymph node
2 = 2+ lymph nodes on same side of diaphragm
3 = both sides of diaphragm
4 = extralymphatic dissemination
Term
suffixes for Hodkin's disease
Definition
A = no constitutional sxs
B = weight loss, night sweats, - presence of constitutional sxs worsens prognosis
Term
bimodal distribution of Hodgkin Disease
Definition
15-30 years
> 50 years
Term
clinical featurse of Hodgkin Lymphoma
Definition
painless LAD
supraclavicular, cervical, axillary, mediastinal lymph nodes
spreads continuously from one lymph node to an adjacent lymph node
Term
dx of Hodgkin Lymphoma
Definition
Reed Sternberg cells (look like owl eyes)
presence of inflammatory cell infiltrates (distinguishes from NHL)
CXR and CT to detect lymph node involvement
boen marrow biopsy
labs - leukocytosis, eosinophilia, ESR
Term
tx of Hodgkin disease
Definition
chemo and radiation therapy
Term
risk factors for NHL
Definition
HIV/AIDS
immunosuppression
hx of viral infection
hx of H Pylori gastritis
autoimmune disease
Term
dx of NHL
Definition
lymph node biopsy >1cm for > 4wks
CXR - hilar or mediastinal adenopathy
Term
tx of intermediate and high grade NHL
Definition
CHOP therapy
Cyclophosphamide
Hydroxydaunomycin (doxorubicin)
Oncovin (vincristine)
prednisone
Term
MC malignancy in children under age 15
Definition
ALL
Term
myelogenous leukemia affects what cells
Definition
granulocytes or monocytes
Term
leukemia with testicular involvement
Definition
ALL
Term
leukemia with skin nodules
Definition
AML
Term
auer rods are pathognomonic for
Definition
AML
Term
MC leukemia after age 50
Definition
CLL
Term
smude cells are pathognomonic for
Definition
CLL
Term
tumor lysis syndrome
Definition
potential complication of chemotherapy seen in acute leukemia and high grade NHL.
rapid cell death with release of intracellular contects caueses hyperkalemia, hyperphosphatemia, and hyperuricemia.
treat as a medical emergency
Term
philadelphia chromosome
Definition
CML
Term
diagnostic criteria for polycythemia vera
Definition
must have all 3 major plus any 2 minor
Major: elevated RBC mass, O2 sat >92%, splenomegaly
Minor: thrombocytosis (>400), leukocytosis (>12), leukocyte alk phos (>100), serum B12 (>900)
Term
tx of polycythemia vera
Definition
repeated phlebotomy
myelosupprsesion with hyroxyurea or recombinant interferon alfa
Term
secondary causes of polycythemia vera (2)
Definition
hypoxemia
carbon monoxide exposure
Term
dx of myelodysplastic syndromes
Definition
bone marrow biopsy - blasts or ringed sideroblasts
Howell Jolly Bodies
Term
tx of mylodysplastic syndromes
Definition
mainly supportive
RBC and platelet transfusions
erythropoeitin
granulocyte colony stimulating factor
vitamin supplementation, B6, B12 and folate
Pharm therapies - immunosuppression, cehmo, androgenic steroids
bone marrow transplant = only potential cure
Term
definition of essential thrombocytosis
Definition
> 600,000 platelets
Term
lab findings in thrombocytosis
Definition
splenomegaly, pseudohyperkalemia, elevated bleedng time
bone marrow biopsy - megakaryocytes
smear = hypogranular, abnormally shaped platelets
Term
tx of thrombocytosis
Definition
antiplatelet agents - anagrelide and low dose aspirin
hydroxyurea is sometimes used if severe
Term
hallmark feature of agnogenic myeloid metaplasia with myelofibrosis
Definition
teardrop cells on peripheral smear
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