Hematology Flashcards

1
Q

What is the receptor on platelets that binds to vWF?

A

GP Ib

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2
Q

What is the receptor on platelets that binds other platelets and fibrinogen?

A

GP IIa/IIIb

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3
Q

What are the five major causes of eosinophilia?

A
Neoplasms
Asthma
Allergic processes
CT diseases
Parasites (NAACP)
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4
Q

What are the histological features of neutrophils?

A

Multilobed nuclei

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5
Q

Hypersegmented nuetrophils are seen in what condition?

A

b12 deficiency (pernicious anemia)

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6
Q

What is the function of basophils?

A

Cells that contain heparin and histamine (similar to mast cells)

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7
Q

What is the cause of hemophilia A?

A

Factor 8 deficiency

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8
Q

What is the cause of hemophilia B?

A

Factor 9 deficiency

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9
Q

What are the factors that warfarin inhibits?

A

10 9 7 2 protein S and C

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10
Q

What is the function of protein S and C?

A

C is activated by thrombomodulin/thrombin complex, which in turn activates protein S, and deactivates factors 8 and 5

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11
Q

What is the order of the extrinsic pathway?

A

3
7
10 (+8)

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12
Q

What is the order of the intrinsic pathway?

A

12
11
9
10

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13
Q

What are the factors that comprise the tenase complex? What does this complex do?

A

8 and 9

Increases thrombin activation

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14
Q

What is the MOA of tPA?

A

activates plasminogen to plasmin

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15
Q

What is the MOA of clopidogrel?

A

Prevents ADP receptor binding

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16
Q

What is the MOA of abciximab?

A

Ab against GP IIa/IIIb

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17
Q

What is the MOA of ASA’s antiplatelet effect?

A

inhibits thromboxane A2 synthesis

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18
Q

What is von willebrand disease?

A

Insufficient vWF

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19
Q

Basophilic stippling of RBCs is found in what diseases? (4)

A

anemia of chronic disease
alcohol abuse
Pb poisoning
thalassemias

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20
Q

Bite cells = what disease?

A

G6PD deficiency

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21
Q

elliptocytes = what disease?

A

Hereditary elliptocytosis

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22
Q

Ringed siderblasts = what disease?

A

Sideroblastic anemia (microcytic anemia

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23
Q

What are the five major causes of microcytic anemia?

A
Fe deficiency
Thalassemias
sideroblastic anemia
Anemia of chronic disease
Pb poisoning
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24
Q

Heinz bodies = what disease? What are they?

A

G6PD deficiency

Denatured Hb

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25
Q

Howell-Jolly bodies = what disease?

A

hyposplenia or asplenia

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26
Q

What are schistocytes? What are these seen in?

A

Broken RBCs—DIC, TTP/HUS mechanical heart valves

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27
Q

What are the two major causes of macrocytic anemia?

A

B12 deficiency

Folate deficiency

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28
Q

What is/are the labs that separate B12 deficiency and folate deficiency?

A

Methylmalonic acid will be high in B12 deficiency

29
Q

What is the one reaction in the body that requires THF?

A

Conversion of homocysteine to methionine

30
Q

What is TIBC?

A

The amount of Fe that can be stored on transferrin (the number of open spots, thus higher numbers = lower serum ferritin)

31
Q

What are the two storage forms of Fe?

A

Ferritin (dynamic)

Hemosiderin (lost)

32
Q

What is plummer vinson syndrome?

A

Fe deficiency
Esophageal webs
atrophic glossitis

33
Q

What is the Hb formed when there is are three (or four total) deletions of the alpha globin gene?

A

HbH (beta4 tetramer)

34
Q

What is Bart’s hb? What disease is it found in?

A

gamma4 tetramer of Hb, found in 4 alpha gene deletions

35
Q

What is beta thalassemia minor?

A

underproduction of beta globin. This is usually asymptomatic, since there is an upregulation of HbA2 (alpha2, delta2)

36
Q

What are the components of HbA?

A

alpha2beta2

37
Q

What are the components of HbA2?

A

alpha2delta2

38
Q

What are the components of HbF?

A

Alpha2gamma2

39
Q

Mohawk appearance of a skull x-ray = what disease? Why?

A

beta thalassemia major d/t increased hematopoiesis in the skull bones

40
Q

What virus are people with beta thalassemia major particularly susceptible to?

A

Parvovirus B19 d/t infection of erythroid progenitor cells

41
Q

What are teardrop cells seen in?

A

Primary myelofibrosis

42
Q

What are Target cells seen in? (HALT)

A

HbC disease
Asplenia
Liver dz
Thalassemia

43
Q

What are iron studies like with Fe deficiency anemia? (Fe, TIBC, ferritin)

A

Low Fe
High TIBC
Low Ferritin

44
Q

What is the major toxicity of Pb poisoning?

A

Inhibition of ferrochelatase and ALA dehydratase

45
Q

What are the ssx of Pb poisoning?

A

Lead lines in gingiva

Encephalopathy

46
Q

What is the treatment for Pb poisoning?

A

EDTA or Succimer drops

47
Q

What are the PBS findings for Pb poisoning?

A

Microcytic anemia with basophilic stippling

48
Q

What is the hereditary cause of sideroblastic anemia? What is the inheritance pattern of this?

A

X-linked deficiency/defect i delta-ALA synthase gene

49
Q

What drug classically causes sideroblastic anemia? How? What is the treatment for this?

A

Isoniazid d/t inhibition of ALA synthase

B6 supplementation

50
Q

What are the PBS findings of sideroblastic anemia?

A

Ringed sideroblasts d/t Fe laden mitochondria

51
Q

What are the iron studies like with sideroblastic anemia? (Fe, TIBC, Ferritin)?

A

Increase Fe
Normal TIBC
Increased Ferritin

52
Q

What are homocysteine and methylmalonic acids levels like with folate and B12 deficiencies respectively?

A
B12 = both high
Folate = only Homocysteine levels are elevated
53
Q

What is orotic aciduria, and what causes it? How does this present?

A

Inability to convert orotic acid to UMP because of a effect in UMP synthase

Presents in childhood as megaloblastic anemia that cannot be corrected with folate or B12

54
Q

How do you differentiate orotic aciduria from ornithine transcarbamylase deficiency?

A

No hyperammonemia with orotic aciduria

55
Q

What is non-megaloblastic macrocytic anemia?

A

Macrocytic anemia in which DNA synthesis is not impaired. Usually caused by EtOH-ism or cirrhosis

56
Q

What are the two different types of normocytic, normochromic anemias?

A

Hemolytic vs nonhemolytic

57
Q

What are the following labs like with intravascular hemolysis:

  • Haptoglobin
  • LDH
  • reticulocytes
A

Decreased haptoglobin (is bound)
Increased LDH
-Increased reticulocytes

58
Q

What happens to LDH and unconjugated bili levels in extravascular hemolysis?

A

Increased LDH and increased bili

59
Q

What is the role of hepcidin?

A

binds ferroportin in the intestinal mucosa and macrophages, thus inhibiting Fe uptake

60
Q

What is the pathophysiology of anemia of chronic disease?

A

Chronic inflammation leads to increased hepcidin release, and an increase in ferritin levels, as Fe is preferentially stored away from bacteria

61
Q

What are the following labs like with anemia of chronic disease:

  • Fe
  • TIBC
  • Ferritin
A

Low Fe
Low TIBC
High Ferritin

62
Q

What is the body’s main form of Fe storage?

A

Ferritin

63
Q

How does CKD lead to anemia?

A

Low EPO = low hematopoiesis

64
Q

What is aplastic anemia, and what are the classical findings of it?

A

Pancytopenia, characterized by normal cell morphology, but hypocellular bond marrow with fatty infiltration

65
Q

What is the bone aspiration finding of aplastic anemia?

A

Dry tap (fat cells)

66
Q

What is the treatment for aplastic anemia?

A

Marrow transplant

g-CSF

67
Q

What viruses can cause aplastic anemia? (4)

A

Parvo B19
HIV
HCV
EBV

68
Q

What is the pathophysiology of Fanconi anemia?

A

DNA repair defect, leading to aplastic anemia