17. Heme Flashcards

(72 cards)

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
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