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Step 1 DIT > 17. Heme > Flashcards

17. Heme Flashcards

1
Q

What coagulation factor is deficient in hemophilia A?

A

8

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2
Q

What coagulation factor is deficient in hemophilia B?

A

9

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3
Q

What is the clinical consequence of a deficiency in either protein C or protein S?

A

hypercoagulable

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4
Q

What is the treatment for overdose of heparin?

A

protamine sulfate

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5
Q

What is the treatment for heparin-induced thrombocytopenia?

A
  • stop heparin

- start different anticoagulant

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6
Q

What allows RBCs to change shape as they pass through vessels?

A

spectrin

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7
Q

What name is given to immature erythrocytes in circulation?

A

reticulocytes

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8
Q

What pathologic form of RBC would you see in each of the following diseases?
- lead poisoning

A

basophilic stippling

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9
Q

What pathologic form of RBC would you see in each of the following diseases?
- G6PD deficiency

A

Heinz bodies –> bite cells

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10
Q

What pathologic form of RBC would you see in each of the following diseases?
- DIC

A

schistocytes

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11
Q

What pathologic form of RBC would you see in each of the following diseases?
- abetalipoproteinemia

A

acanthocyte (spur cell)

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12
Q

What pathologic form of RBC would you see in each of the following diseases?
- asplenia

A

Howell-Jolly bodies

Target cells

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13
Q

Where does fetal erythropoiesis take place?

A

Yolk sac –> liver –> spleen –> BM

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14
Q

In which adult bones does erythropoiesis take place?

A

axial skeleton: vertebrae, ribs, pelvis

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15
Q

What are some of the diff. causes of polycythemia? (4)

A
  • ectopic EPO (pheo, RCC, HCC, hemangioblastoma)
  • chronic hypoxia
  • polycythemia vera
  • trisomy 21
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16
Q

What are the hematologic and non-hematologic findings in a patient with lead poisoning?

A

hematologic: microcytic anemia, basophilic stippling

non-heme: encephalopathy, memory loss, HA, foot/wrist drop, lead lines on gingivae, abdominal colic, renal failure

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17
Q

What test can be used to diagnose beta-thalassemia minor?

A

Hb electrophoresis (will see increased HbA2)

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18
Q

What should you r/o in a man over 50 with new-onset iron deficiency anemia?

A

colon cancer

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19
Q

A patient is diagnosed with a macrocytic, megaloblastic anemia. What is the danger of giving folate alone?

A

uncorrected B12 def –> peripheral neuropathy

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20
Q

What is the cause of anemia?

- microcytic anemia + swallowing difficulty + glossitis

A

Plummer Vinson syndrome

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21
Q

What is the cause of anemia?

- microcytic anemia, >3.5% HbA2

A

beta-thal minor

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22
Q

What is the cause of anemia?

- megaloblastic anemia not correctable by B12 or folate

A

orotic aciduria

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23
Q

What is the cause of anemia?

- megaloblastic anemia along with peripheral neuropathy

A

b12 deficiency

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24
Q

What is the cause of anemia?

- microcytic anemia + basophilic stippling

A

lead poisoning

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25
Q

What is the cause of anemia?

- microcytic anemia reversible with B6

A 
sideroblastic anemia 
(note: B6 is cofactor for delta-ALA synthase)
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26
Q

What is the cause of anemia?

- HIV-positive patient with macrocytic anemia

A

zidovudine (HIV drug)

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27
Q

What is the cause of anemia?

- normocytic anemia and elevated creatinine

A

chronic kidney disease

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28
Q

A child anemic since birth has now been cured with splenectomy. What is the disease?

A

hereditary spherocytosis

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29
Q

What findings are associated with hereditary spherocytosis?

A

anemia, jaundice (b/c extravascular), pigmented gall stones, splenomegaly

  • (+) osmotic fragility test
  • (-) Coombs
30
Q

What is the difference b/w hemoglobin S defect and hemoglobin C?

A

HbS (sickle cell) glu –> valine

HbC: glu –> lysine

31
Q

What is the difference b/w warm agglutinin and cold agglutinin

A

warm: IgG
cold: IgM

32
Q

What are schistocytes?

A

fragmented RBCs by mechanical destruction

33
Q

In what hematologic disorder would you find each of the following abnormal tests/findings?
- Heinz bodies

A

G6PD

34
Q

In what hematologic disorder would you find each of the following abnormal tests/findings?
- osmotic fragility test

A

hereditary spherocytosis

35
Q

What is the life span of a platelet (which is also the max life of platelets after a transfusion)?

A

8-10 days

36
Q

What molecule is expressed on the surface of a platelet after it becomes activated?

A

GP IIb/IIIa

37
Q

NSAIDs inhibit the production of which substance important in platelet aggregation?

A

Thromboxane A2

38
Q

What is the MoA of each of the following drugs?

- Streptokinase

A

thrombolytic (converts plasminogen –> plasmin) –> cleaves fibrin

39
Q

What is the MoA of each of the following drugs?

- aspirin

A

irreversibly inhibits COX1 and 2

40
Q

What is the MoA of each of the following drugs?

- clopidogrel

A

blocks ADP receptors

41
Q

What is the MoA of each of the following drugs?

- abciximab

A

binds GP IIb/IIIa on platelets

42
Q

What is the MoA of each of the following drugs?

- tirofiban

A

binds GP IIb/IIIa on platelets

43
Q

What is the MoA of each of the following drugs?

- ticlopidine

A

blocks ADP receptors

44
Q

What is the MoA of each of the following drugs?

- enoxaparin

A

LMWH (inhibit factor 10a)

45
Q

What is the MoA of each of the following drugs?

- eptifibatide

A

GP IIb/IIIa inhibitor

46
Q

What is the cause of ITP?

A

antibodies to glycoprotein IIb/IIIa on platelets (which then gets removed from circulation)

47
Q

What is the defect in Bernard-Soulier disease?

A

deficient GP1b –> defect in plt-to-vWF adhesion

48
Q

After a normal spontaneous vaginal delivery, the new mom bleeds profusely from her vagina and later from her gums. What abnormal lab values would you suspect?

A
  • decreased PC
  • increased BT, PT, PTT
  • increased d-dimer, decreased fibrinogen
49
Q

Compare the age distribution of those affected by Hodgkin lymphoma to those affected by non-Hodgkin lymphoma

A

HL: bimodal (peak @ age 20, 65)
NHL: variable

50
Q

What is the most common type of non-Hodgkin lymphoma in adults? In children?

A

adults: diffuse large B cell lymphoma
children: lymphoblastic lymphoma

51
Q

What form of lymphoma matches each of the following statements?
- most common lymphoma in US

A

diffuse large B-cell lymphoma

52
Q

What form of lymphoma matches each of the following statements?
- reed-sternberg cells

A

hodgkin lymphoma

53
Q

What form of lymphoma matches each of the following statements?
- particularly associated with EBV

A

Burkitt lymphoma, hodgkin lymphoma

54
Q

What form of lymphoma matches each of the following statements?
- associated with long-term celiac disease

A

intestinal T-cell lymphoma

55
Q

What form of lymphoma matches each of the following statements?
- lymphoma equivalent of CLL

A

small lymphocytic lymphoma

56
Q

What form of lymphoma matches each of the following statements?
- “starry sky” pattern due to phagocytosis of apoptotic tumor cells

A

burkitt

57
Q

What form of lymphoma matches each of the following statements?
- associated with sjogren syndrome, hashimoto thyroiditis, and H. pylori

A

marginal cell MALToma

58
Q

A patient presents with anemia, hypercalcemia, and bone pain undergoes a bone marrow biopsy, which reveals plasma cells. What is the diagnosis and what may be found on urinalysis?

A

Multiple myeloma

  • no proteinuria on standard UA
  • need UPEP for detection of Bence Jones proteins
59
Q

What form of leukemia matches each of the following?

- most common leukemia in children

A

ALL

60
Q

What form of leukemia matches each of the following?

- most common leukemia in adults in US

A

CLL

61
Q

What form of leukemia matches each of the following?

- characteristic Auer rods

A

AML (M3 type)

62
Q

What form of leukemia matches each of the following?

- myelodysplastic syndromes have a tendency to progress to ___

A

AML

63
Q

What form of leukemia matches each of the following?

- myeloproliferative disorders may progress to ___

A

AML

64
Q

What form of leukemia matches each of the following?

- more than 20% blasts in marrow

A

acute leukemia

65
Q

What form of leukemia matches each of the following?

- leukemia with more mature cells and

A

chronic leukemia

66
Q

What form of leukemia matches each of the following?

- TdT (+) acute leukemia

A

ALL

67
Q

What form of leukemia matches each of the following?

- commonly presents with bone pain

A

ALL

68
Q

What form of leukemia matches each of the following?

- numerous basophils, splenomegaly, and negative for leukocyte alkaline phosphatase

A

CML

69
Q

What form of leukemia matches each of the following?

- always positive for the philadelphia chromosome (t9;22)

A

CML

70
Q

What form of leukemia matches each of the following?

- acute leukemia positive for peroxidase

A

AML

71
Q

What form of leukemia matches each of the following?

- solid sheets of lymphoblasts in marrow

A

ALL

72
Q

What form of leukemia matches each of the following?

- always associated with the bcr-abl gene

A

CML

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