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Heme/Lymph > Exam > Flashcards

Exam Flashcards

1
Q

ddx: spherocytes (3)

A

hereditary spherocytosis, G6PDH deficiency, warm/cold AIHA

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2
Q

bite cells

A

G6PDH deficiency

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3
Q

ddx: schistocytes (4)

A

TTP, DIC, HUS, malignant hypertension

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4
Q

treatment for sickle cell anemia; mechanism

A

hydroxyurea; increases levels of HbF

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5
Q

chipmunk facies

A

beta-thalassemia major

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6
Q

which thalassemia: virtually all HbF

A

beta-thalassemia major

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7
Q

which thalassemia: elevated HbA2

A

beta-thalassemia minor/beta-thalassemia trait

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8
Q

which thalassemia: basophilic stippling

A

beta-thalassemia minor

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9
Q

which thalassemia: HbH

A

alpha-thalassemia with 3 alpha genes deleted

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10
Q

inheritance of hereditary spherocytosis

A

autosomal dominant

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11
Q

ddx: aplastic crises caused by parvovirus

A

sickle cell anemia and hereditary spherocytosis

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12
Q

thalassemias: ____vascular hemolysis

A

extra

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13
Q

inheritance of G6PDH deficiency

A

X-linked

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14
Q

Heinz bodies (accumulation of denatured, precipitated hemoglobin)

A

G6PDH deficiency

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15
Q

PNH: mutated gene

A

PIGA

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16
Q

mutation in PIGA causes what mechanism of disease?

A

decreased GPI-linked proteins including anti-complement proteins CD55 and CD59

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17
Q

warm AIHA: Ig__ antibodies, ____vascular hemolysis in _____

A

G, extra, spleen

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18
Q

cold AIHA: Ig__ antibodies, ____vascular hemolysis in _____

A

M, extra (can also be intravascular), liver

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19
Q

hemolytic anemia that happens 2-3 weeks into EBV or Mycoplasma infections

A

acute form of cold AIHA

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20
Q

what in the spleen stains red?

A

blood-filled sinusoids

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21
Q

what in the spleen stains blue?

A

cords of densely packed lymphocytes between sinusoids

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22
Q

RBC infiltrate in post-splenectomy patient

A

Howell Jolly bodies

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23
Q

explain severe vasoconstriction and hypertension in hemolysis

A

free hemoglobin scavenges NO

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24
Q

CD163 scavenger receptor: binds what, where is it expressed

A

haptoglobin-hemoglobin complex; monocyte/macrophage lineage (recycles between early endosomes and the plasma membrane)

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25
Q

what two things happen when haptoglobin’s buffering capacity is overwhelmed?

A
  1. hemoglobin converts to metHb, liberating heme

2. free heme binds to albumin and subsequently transferred to hemopexin

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26
Q

heme ring opening is catalyzed by what enzyme and what are the 3 products?

A

HO-1; bilverdin, CO and iron (iron is bound by ferritin and CO is bound by hemoglobin)

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27
Q

bilirubin is ______ than biliverdin

A

less polar

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28
Q

microcytic, hypochromic anemia with pronounced anisopoikiloytosis

A

iron deficiency anemia

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29
Q

TIBC is qualitatively the opposite of what?

A

serum ferritin

30
Q

iron is absorbed where? what affects this part of the small intestine?

A

duodenum; Crohn’s

31
Q

what molecule is excess iron stored in during iron overload?

A

hemosiderin

32
Q

hypersegmented neutrophils

A

megaloblastic anemia

33
Q

macrocytes, macroovalocytes, anisopoikilocytosis

A

megaloblastic anemia

34
Q

nuclear to cytoplasmic asynchrony in bone marrow

A

megaloblastic anemia

35
Q

what enzyme in the folate pathway is inhibited by methotrexate?

A

dihydrofolate reductase (converts folate into TH4)

36
Q

what enzyme in the folate pathway is inhibited by 5-FU?

A

thymidylate synthetase

37
Q

why does B12 deficiency cause megaloblastic anemia?

A

B12 is a cofactor for methionine synthase, the only enzyme that can regenerate TH4

38
Q

where is folate absorbed?

A

jejunum

39
Q

where is B12 absorbed?

A

ileum

40
Q

mechanism of pernicious anemia

A

autoantibodies against intrinsic factor, causes B12 deficiency –> megaloblastic anemia

41
Q

what features distinguish B12 deficiency from folate deficiency?

A

B12 deficiency causes neurologic deficits and will show elevated methylmalonic acid

42
Q

what disorder is caused be defect in MPL gene?

A

congenital amegakaryocytic thrombocytopenia

43
Q

congenital amegakaryocytic thrombocytopenia: defect in what gene?

A

MPL

44
Q

inherited bone marrow syndrome causing pancreas and bone marrow deficiency

A

Schwachman-Diamond syndrome

45
Q

what does eltrombopag do?

A

thrombopoietin agonist = increases platelets

46
Q

abnormal finger/toenails

A

dyskeratosis congenita

47
Q

3 effects of telomere diseases

A

bone marrow failure, liver fibrosis and lung fibrosis

48
Q

which factor activates factor 2 into initial thrombin?

A

factor 10a

49
Q

what does initial thrombin activate?

A

Factors 5, 8, 11 and platelets

50
Q

vitamin K dependent factors

A

Factors 2, 7, 9, 10, Protein C and Protein S

51
Q

antecedent viral syndrome, abrupt onset of severe thrombocytopenia

A

childhood ITP

52
Q

treatment for refractory ITP

A

IVIG, prednisone, splenectomy, thrombopoietin agonists, rituximab

53
Q

pentad of TTP

A

microangiopathic hemolytic anemia, thrombocytopenia, renal failure, mental status changes, fever

54
Q

TTP: mechanism of disease

A

congenital or acquired autoantibody against ADAMTS13

55
Q

Bernard Soulier syndrome: mechanism of disease

A

congenital absence of GpIb

56
Q

Glanzman’s thrombasthenia: mechanism of disease

A

congenital absence of GpIIb/IIIa

57
Q

difference between PT and PTT

A

PT: add tissue factor
PTT: add surface activator

58
Q

mechanism of heparin

A

causes conformational change in antithrombin, accelerating its inhibition of thrombin and Factor 10a

59
Q

Factors not tested by screens

A

Factor 8, antithrombin, Protein C, Protein S

60
Q

liver disease: what additional factors are deficient on top of those that are vitamin K dependent?

A

Factor 5 and antithrombin

61
Q

what factors are digested by Protein C?

A

Factors 5 and 8

62
Q

how does thrombomodulin work?

A

it is bound to endothelial cell surface, binds thrombin and turns it into an ANTIcoagulant enzyme which then activates Protein C –> inhibits Factors 5 and 8

63
Q

why does Factor 5 Leiden have tendency for venous thrombosis?

A

impaired cleavage of Factor 5 by Protein C

64
Q

venous and arterial thrombosis, pregnancy complication associated with fetal demise

A

APLAS (antiphospholipid antibody syndrome)

65
Q

treatment of vWD

A

desmopressin (synthetic analog of vasopressin; releases vWF from endothelial storage)

66
Q

ddx: decreased fibrinogen

A

DIC, liver disease

67
Q

ddx: isolated prolonged PTT

A

hemophilias, APLAS

68
Q

which transfusion-related adverse effects present with hypertension? hypotension?

A

hypertension: FNHTR, TACO
hypotension: TRALI

69
Q

which transfusion-related adverse effects present with fever?

A

FNHTR, hemolytic transfusion reaction, TRALI

70
Q

red urine after transfusion

A

hemoglobinuria due to acute hemolytic transfusion reaction (ABO errors)

71
Q

what conditions can cause arterial AND venous thromboses?

A

heparin-induced thrombocytopenia and APLAS

Heme/Lymph (3 decks)

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