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USMLE 3 - Hematology
questions from First aid for USMLE step 3
57
Medical
Professional
08/22/2009

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Term
What labratory tests should be ordered for anemic pts?
Definition
CBC, MCV, blood smear, reticulocyte count
Term
What type of blood matching tests should be ordered for pts with significant bleeds?
Definition
cross and type
Term
Anemias with MCV values below 70 are of what to types?
Definition
Thalassemia or iron-deficiency
Term
What vales of serum ferritin suggest iron-deficiency anemia?
Definition
decreased
Term
Alpha-thalassemia is common to what two ethnic groups?
Definition
Asians and African-Americans
Term
Beta-thalasseemia (In addition to being in Asians and African-Americans) is more common in what ethnic group?
Definition
Mediteraneans
Term
What are the TICS (mneumonic) for causes of microcytic anemia?
Definition
Thalassemia
Iron deficiency
Chronic disease
Sideroblastic anemia
Term
Which two types of anemia are considered for MCV less than 70?
Definition
iron-deficiency
thalasesemia
Term
What is hydrops fetalis?
Definition
prenatal form of third-spacing as a result of heart failure
Term
A fetus with four alleles of alpha-thalassemia results in what?
Definition
hydrops fetalis (abortion, or still born)
Term
How do pts with only one or two alleles for alpha-thalessemia present clinically?
Definition
asymptomatic
Term
How is beta-thalassemia major diagnosed?
Definition
complete lack of HbA
Term
How is beta-thalassemia minor diagnosed?
Definition
increased HbA2 by gel electrphoresis
Term
What treatment is used in the cause of transfusion overload?
Definition
iron chelator (deferoxamine)
Term
With MCV values over 110 what are the two most likely causes?
Definition
Vit B12 deficiency
folate deficiency
Term
What are the CBC, RDW, and RBC characteristics of the thalassemias?
Definition
CBC is microcytic
RDW is normal
RBC is increased
Term
What treatments are viable for thalassemia pts?
Definition
allogenic bone marrow transplant with folate supplementation
Term
What is the Schilling test in hematology?
Definition
study combining IM and oral B12 supplementation to determine cause of megaloblastic anemia
Term
What role does intrinsic factor play in anemia?
Definition
produced by parietal cells of stomach to facilitate B12 absorption (pts with megaloblastic anemia should be tested for intrinsic factor antibody)
Term
When is methylmalonic acid used in the evaluation of anemia?
Definition
to help determine if megaloblastic anemia is due to B12 deficiency
Term
How should B12 deficiency be treat? How about folate deficiency?
Definition
Monthly injections
folate is supplemented orally
Term
What autoimmune test is negative in cases of hereditary spherocytosis?
Definition
Coomb's test
Term
When should cold agglutinin antibodies be tested for?
Definition
in pts with evidence of hemolysis without other explanations
Term
What drug class is particularly well known to cause a hemolytic episode in G6PD pts?
Definition
sulfa drugs
Term
Descrube G6PD levels in G6PD deficient pts both in a hemolytic crisis and when there is no hemolytic crisis?
Definition
Hemolysis: lvls may appear normal
non-hemolysis situation: lvls are low/depleted
Term
How is paroxysmal nocturnal hemoglobinuria diagnosed?
Definition
flow cytometry
Term
What is different about the genetics of paroxysmal nocturnal hemoglobinuria?
Definition
it's an acquired genetic mutation rather than inheritted
Term
What is the treatment of choice for pts with hereditary sherocytosis?
Definition
splenectomy
Term
What are the three characteristics of hemolytic uremic syndrome?
Definition
hemolysis
renal failure
thrombocytopenia
Term
What are the five characteristics of thrombotic thrombocytopenic purpura?
Definition
HUS (hemolysis, renal failure, thrombocytopenia)
fever
fluctuating neurologic sings
Term
What type of bodies are seen in pts with sickle cell anemia?
Definition
Howell-Jolly bodies
Term
Which three vaccinations should all sickle cell pts receive?
Definition
Strep pneumoniae
H. influenzae
HBV
Term
What is the role of hydroxurea in managing sickle cell disease?
Definition
serves to decrease number and severity of sickle crisis and increases circulating fetal hemoglobin
Term
A pt presenting to the ER in sickle crisis should have what three factors attended immediately?
Definition
hydration
analgesia
supplemental oxygen
Term
What virus is well known to cause aplastic crisis in sickle cell pts?
Definition
Parvovirus B19
support with transfusions
Term
Which antibiotics are commonly used in the treatment of sickle cell pts with suspicion of infection?
Definition
2nd gen cephalosporin plus erythromycin
Term
What are the four big infections common to sickle cell pts?
Definition
Strep pneumoneae
H. influenzae
Neisseria meningitidis
Gram-neg bugs
Term
What is the classic age group for polycythemia vera?
Definition
male pt over 60
Term
What is plethora?
Definition
large retinal veins on funduscopy
Term
What hormone can be evaluated to differentiated secondary erythrocytosis from polycythemia vera?
Definition
erythropoietin (which is low in PCV with elevated RBC and hematocrit)
Term
Once polycythemia vera is suspected what is done to clench diagnosis?
Definition
bone marrow biopsy
Term
What part of blood should be suspected of being deficient when petechiae are present?
Definition
platelets
Term
What are the three components of DIC blood profile?
Definition
D-dimer
fibrinogen
blood smear
Term
What serum protein is deficient in a pt with an extended thrombin time?
Definition
fibrinogen
Term
Bleeding time measures what?
Definition
platelet aggregation
Term
How is bleeding clinically different in platelet disorders vs. coagulopathies?
Definition
platelet: excessive, prolonged bleeding which is immediately apparant
coagulopathy: slightly increased time for bleed, late bleed after trauma or surgery
Term
Which type of thrombocytopenia is due to acutely formed platelet-associated IgG antibodies?
Definition
ideopathic thrombocytopenic purpura
Term
What labs are ordered for a supposed case of DIC?
Definition
PT (sig. prolonged)
PTT (sig. prolonged)
D-dimer (elevated)
fibrinogen (low)
Term
How many days after initiating heparin does heparin-induced thrombocytopenia usually manifest?
Definition
4-14 days
Term
What antibody is measure to make the diagnosis of heparin-induced thrombocytopenia?
Definition
PF-4 (platelet factor 4)
Term
What lab should be ordered to diagnose the most common inherited bleeding disorder (hemophilia)?
Definition
PTT
Term
Blood factor II is known by what other name?
Definition
fibrinogen
Term
Which factors are in the common pathway?
Definition
II, V, X
Term
When should labs be ordered to assess factors II, V, and X?
Definition
When coagulopathy is present and both PT and PTT are elevated without evidence of heparin use, DIC, liver disease, or Vit K deficiency
Term
Which factor is deficient in hemophilia B?
Definition
Factor IX
Term
What disease is the most common inheritable hypercoagulable disorder?
Definition
Factor V Leiden deficiency (in which factor V cannot be inactivated by protein C)
Term
How long should heparin be continued after initiating anticoagulation treatment?
Definition
until INR reaches therapeutic levels (usually 3-5 days)
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