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Flashcards in 17. Heme Deck (72):
1

What coagulation factor is deficient in hemophilia A?

8

2

What coagulation factor is deficient in hemophilia B?

9

3

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

hypercoagulable

4

What is the treatment for overdose of heparin?

protamine sulfate

5

What is the treatment for heparin-induced thrombocytopenia?

- stop heparin
- start different anticoagulant

6

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

spectrin

7

What name is given to immature erythrocytes in circulation?

reticulocytes

8

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

basophilic stippling

9

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

Heinz bodies --> bite cells

10

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

schistocytes

11

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

acanthocyte (spur cell)

12

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

Howell-Jolly bodies
Target cells

13

Where does fetal erythropoiesis take place?

Yolk sac --> liver --> spleen --> BM

14

In which adult bones does erythropoiesis take place?

axial skeleton: vertebrae, ribs, pelvis

15

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

- ectopic EPO (pheo, RCC, HCC, hemangioblastoma)
- chronic hypoxia
- polycythemia vera
- trisomy 21

16

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

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

17

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

Hb electrophoresis (will see increased HbA2)

18

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

colon cancer

19

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

uncorrected B12 def --> peripheral neuropathy

20

What is the cause of anemia?
- microcytic anemia + swallowing difficulty + glossitis

Plummer Vinson syndrome

21

What is the cause of anemia?
- microcytic anemia, >3.5% HbA2

beta-thal minor

22

What is the cause of anemia?
- megaloblastic anemia not correctable by B12 or folate

orotic aciduria

23

What is the cause of anemia?
- megaloblastic anemia along with peripheral neuropathy

b12 deficiency

24

What is the cause of anemia?
- microcytic anemia + basophilic stippling

lead poisoning

25

What is the cause of anemia?
- microcytic anemia reversible with B6

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

26

What is the cause of anemia?
- HIV-positive patient with macrocytic anemia

zidovudine (HIV drug)

27

What is the cause of anemia?
- normocytic anemia and elevated creatinine

chronic kidney disease

28

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

hereditary spherocytosis

29

What findings are associated with hereditary spherocytosis?

anemia, jaundice (b/c extravascular), pigmented gall stones, splenomegaly
- (+) osmotic fragility test
- (-) Coombs

30

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

HbS (sickle cell) glu --> valine
HbC: glu --> lysine

31

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

warm: IgG
cold: IgM

32

What are schistocytes?

fragmented RBCs by mechanical destruction

33

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

G6PD

34

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

hereditary spherocytosis

35

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

8-10 days

36

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

GP IIb/IIIa

37

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

Thromboxane A2

38

What is the MoA of each of the following drugs?
- Streptokinase

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

39

What is the MoA of each of the following drugs?
- aspirin

irreversibly inhibits COX1 and 2

40

What is the MoA of each of the following drugs?
- clopidogrel

blocks ADP receptors

41

What is the MoA of each of the following drugs?
- abciximab

binds GP IIb/IIIa on platelets

42

What is the MoA of each of the following drugs?
- tirofiban

binds GP IIb/IIIa on platelets

43

What is the MoA of each of the following drugs?
- ticlopidine

blocks ADP receptors

44

What is the MoA of each of the following drugs?
- enoxaparin

LMWH (inhibit factor 10a)

45

What is the MoA of each of the following drugs?
- eptifibatide

GP IIb/IIIa inhibitor

46

What is the cause of ITP?

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

47

What is the defect in Bernard-Soulier disease?

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

48

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?

- decreased PC
- increased BT, PT, PTT
- increased d-dimer, decreased fibrinogen

49

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

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

50

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

adults: diffuse large B cell lymphoma
children: lymphoblastic lymphoma

51

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

diffuse large B-cell lymphoma

52

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

hodgkin lymphoma

53

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

Burkitt lymphoma, hodgkin lymphoma

54

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

intestinal T-cell lymphoma

55

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

small lymphocytic lymphoma

56

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

burkitt

57

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

marginal cell MALToma

58

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?

Multiple myeloma
- no proteinuria on standard UA
- need UPEP for detection of Bence Jones proteins

59

What form of leukemia matches each of the following?
- most common leukemia in children

ALL

60

What form of leukemia matches each of the following?
- most common leukemia in adults in US

CLL

61

What form of leukemia matches each of the following?
- characteristic Auer rods

AML (M3 type)

62

What form of leukemia matches each of the following?
- myelodysplastic syndromes have a tendency to progress to ___

AML

63

What form of leukemia matches each of the following?
- myeloproliferative disorders may progress to ___

AML

64

What form of leukemia matches each of the following?
- more than 20% blasts in marrow

acute leukemia

65

What form of leukemia matches each of the following?
- leukemia with more mature cells and

chronic leukemia

66

What form of leukemia matches each of the following?
- TdT (+) acute leukemia

ALL

67

What form of leukemia matches each of the following?
- commonly presents with bone pain

ALL

68

What form of leukemia matches each of the following?
- numerous basophils, splenomegaly, and negative for leukocyte alkaline phosphatase

CML

69

What form of leukemia matches each of the following?
- always positive for the philadelphia chromosome (t9;22)

CML

70

What form of leukemia matches each of the following?
- acute leukemia positive for peroxidase

AML

71

What form of leukemia matches each of the following?
- solid sheets of lymphoblasts in marrow

ALL

72

What form of leukemia matches each of the following?
- always associated with the bcr-abl gene

CML