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IBI Test 3 > B3-066 CBCL Thrombocytopenia > Flashcards

B3-066 CBCL Thrombocytopenia Flashcards

1
Q

small non-nucleated blood cells derived from bone marrow megakaryocytes

A

platelets

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2
Q

alpha granules

A

platelet factor V
fibrinogen
VWF
PDGF

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3
Q

dense granules

A

ADP
serotonin
calcium

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4
Q
A

normal peripheral blood smear

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5
Q
A

normal bone marrow

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6
Q

Gplb deficiency

A

Bernard-Soulier

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7
Q

GpIIb-IIIa deficiency

A

Glanzmann

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8
Q

platelet function tests

A

PFA-100
platelet aggregation test
bleeding test

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9
Q

test of platelet function that measures both platelet adhesion and aggregation

A

PFA test

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10
Q

Normal CT Collagen-ADP
elevated CT Collagen EPI

A

aspirin/NSAIDs

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11
Q

Normal or elevated CT Collagen-ADP
normal or elevated CT Collagen EPI

A

clopidogrel

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12
Q

elevated CT Collagen-ADP
elevated CT Collagen EPI

3

A

VWD
Bernard Soulier
Glanzmann

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13
Q

measures the ability of various agonists to platelets to induced in vitro activation and platelet-platelet activation

A

LTA

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14
Q

LTA: when agnoist is added

A

platelets aggregate and absorb less light

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15
Q

LTA: aggregation present only with ristocetin

A

glanzmann

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16
Q

LTA: aggregation is absent with high concentrations of ristocetin

A

Bernard Soulier

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17
Q

causes of thrombocytopenia

A

decreased platelet production
decreased platelet survival
sequestration in spleen
dilution

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18
Q
A

thrombocytopenia

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19
Q
A

aplastic anemia

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20
Q
A

metastatic carcinoma

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21
Q
A

ITP

foamy histiocytes in spleen

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22
Q
A

TTP

shistocytes

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23
Q

symptoms of thrombocytopenia

A

petechiae
ecchymoses
gum/nose bleeding
GI bleeding, hematuria, excessive menstrual flow

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24
Q

inherited platelet disorders

A

bernard-soulier
glanzmann
disorders of platelet sectretion

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25
Q

defective adhesion

A

bernard-soulier

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26
Q

defective platelet aggregation

A

glanzmann

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27
Q

acquired defects of thrombocytopenia

A

aspirin
uremia

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28
Q

typically occuring in females

A

TTP

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29
Q

typically occuring in children

A

HUS

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30
Q

inhibition/deficiency of ADAMTS13 causes decreased degradation of vWF multimers

A

TTP

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31
Q

triad of thrombocytopenia, MAHA, and acute kidney injury

A

TTP and HUS

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32
Q

triad+ fever+ neurologic symptoms

A

TTP

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33
Q

triad+ blood diarrhea

A

HUS

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34
Q

normal PTT and PT

disease processes

A

HUS/TTP

distinguishes from DIC

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35
Q

treatment TTP

A

plamaphoresis
glucocorticoids
rituximab

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36
Q

treatment HUS

A

supportive

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37
Q

in patients with renal failure, uremic toxins accumulate and interfere with platelet adhesion

A

uremic platelet dysfunction

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38
Q

destruction of platelets in spleen
anti GpIIb/IIIa antibodies cause splenic macrophages to injest platelets

A

ITP

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39
Q

may by idiopathic or secondary to autoimmune disorders

A

ITP

SLE, HIC, HCV, CLL

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40
Q

increased megakaryocytes on bone marrow biopsy, decreased platelet count

A

ITP

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41
Q

treatment ITP

A

glucocorticoids
IvIg
rituximab

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42
Q

blood smear shows no platelet clumping

A

glanzmann

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43
Q

prolonged PT and PTT
decreased platelets
decreased fibrinogen
elevated D dimer

A

DIC

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44
Q

thrombocytopenia and anti-platelet antibodies

A

ITP

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45
Q

what clinical feature is most likely to be seen in ITP?

A

petechiae

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46
Q

classic pentad of TTP

A

fever
thrombocytopenia
MAHA
neurologic abnormalities
renal failure

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47
Q

TTP is caused by autoantibodies that inhibit

A

ADAMTS13

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48
Q

vWF cleaving protease

A

ADAMTS13

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49
Q

anti-ADAMTS13 antibodies cause

A

TTP

50
Q

anti beta 2 glycoprotein I antibodies cause

A

antiphospholipid antibody syndrome

51
Q

anti factor VIII antibodies cause

A

acquired factor VIII deficiency

52
Q

anti-platelet 4 antibodies cause

A

HIT

53
Q

anti-red cell antibodies cause

A

AIHA

54
Q

Glanzmanns does not cause

A

thrombocytopenia

normal platelet count

55
Q

4 causes of thrombocytopenia

A

splenomegaly
aplastic anemia
ITP
DIC

56
Q

What virus can cause
marrow suppression
autoimmune antibodies to GpIIb-IIIa
deposition of immune complexes on platelets
thrombotic microangiopathy

A

HIV

57
Q

diseases due to defective platelet function

4

A

glanzmanns
uremia
bernard soulier
storage pool diseases

58
Q

immune mediated platelet destruction

A

ITP

59
Q

metastatic carcinoma
aplastic anemia
acute myeloid leukemia
myelodysplastic syndromes

would all cause…..

A

thrombocytopenia

60
Q

grey platelets

A

storage pool diseases

61
Q

large platelets on peripheral blood smear
shistocytes on peripheral blood smear
microthrombi in glomerular arterioles
increased megakaryocytes in marrow

A

HUS

62
Q

HUS is commonly caused by …… in children

A

Shiga toxin

63
Q

most effective treatment for HUS

A

supportive care

64
Q

most common pathogen causing HUS

A

E. coli O157:H7

65
Q

HIT is caused by

A

IgG antibodies that bind to multimolecular complexes of platelet factor 4 bound to heparin

66
Q

absence of shistocytes on blood smear excludes

A

aHUS and TTP

67
Q

normal coagulation tests exclude

A

vWD

68
Q

negative medication history excludes

A

drug induced thrombocytopenia

69
Q

ITP is a diagnosis by

A

exclusion

70
Q

autoantibodies against GIIb-IIIa or Ib-IX cause

A

ITP

71
Q

treatment for chronic ITP in adults

A

splenectomy
glucocorticoids
IvIg
rituximab

72
Q
A

small lymphocytes with mature and condensed chromatin, smudge cell= CLL
ITP is commonly associated with CLL

73
Q

commonly associated with ITP

cancer

A

CLL

74
Q

mucosal bleeding is most characteristic of a

A

platelet disorder

75
Q

absent platelet aggregation in response to bovine or human vWF + ristocetin

A

bernard soulier

76
Q

abnormal GPIb-IX

A

bernard soulier

77
Q

giant platelets with thrombocytopenia

A

bernard soulier

78
Q

abnormal platelet aggregation in response to ADP, collagen, and epinephrine

A

glanzmanns

79
Q

abnormal gpIIb-IIIa

A

glanzmanns

80
Q

thrombocytopenia
fever
renal disease
MAHA
neurologic complications

hallmarks of

A

TTP

81
Q

in qualitative platlet dysfunction bleeding time is

A

prolonged

82
Q

platelet dysfunction causes what type of bleeding?

A

bruises, petechiae, epistaxis

83
Q

uremia causes [quantitative or qualitative] platelet dysfunction

A

qualitative

84
Q

trauma causes [quantitative/qualitative] platelet dysfunction

A

qualitative

85
Q

bernard soulier is a [qualitative/quantitative] platelet dysfunction

A

qualitative

86
Q

inheritance pattern of bernard soulier

A

autosomal recessive

87
Q

caused by deficiency of gp1b receptors

A

bernard soulier

88
Q

responsible for adhering platelets to vWF

A

gp1b

89
Q

absence of bp1b in bernard soulier causes

A

no platelet formation, bleeding

90
Q

giant platelets in blood

A

bernard soulier

91
Q

ristocein test is negative

A

bernard soulier

92
Q

glanzmann is a [quantitative or qualitative] platelet dysfunction

A

qualitative

93
Q

inheritance pattern glanzmann

A

autosomal recessive

94
Q

in ristocetin test for bernard-soulier, addition of normal serum does

A

NOT lead to platelet aggregation

95
Q

dysfunctional gp2a3b

A

glanzmann

96
Q

the dysfunctional gp3a2b in glanzmann causes

A

fibrinogen to be unable to bind –> reduced formation of platelet plug –> bleeding

97
Q

thrombocytopenia is defined as a platelet count below

A

150,000

98
Q

bleeding risk with thrombocytopenia doesnt begin until platelets fall below

A

50,000

99
Q

spontaneous bleeding may occur when platelets fall below

A

20,000

100
Q

how do quanitative disorders affect PT/PTT?

A

increase

101
Q

how do qualitative platelet disorders affect PT/PTT?

A

normal

102
Q

caused by IgG autoantibodies against gp2b3a

A

ITP

103
Q

IgG coated platelets in ITP are

A

phagocytsed by macrophages in the spleen

104
Q

ITP in children is usually

A

self limited

105
Q

ITP in adults causes

A

chronic disease with acute flares

106
Q

typically ITP affects

A

young women in 20s-30s

107
Q

treatment of ITP

A

immunosuppresion, corticosteroids, IVIg

108
Q

splenectomy is used to treat

A

refractory ITP

109
Q

caused by development of autoanitbodies that bind platelet factor 4 heparin complex

A

HIT

110
Q

antibody binding of platelets in HIT causes

A

degranulation

release of cytokines, PF4 and ADP –>clotting

111
Q

platelet activation in HIT leads to

A

platelet aggregation and clot formation

DVT, PE

112
Q

phagocytosis of IgG covered platelets in spleen

A

HIT, ITP

113
Q

fever, chills, tachycardia, dyspnea

A

HIT

114
Q

treatment of HIT

A

direct thrombin inhibitor

bivalrudin, argatroban

115
Q

cause diffuse platelet activation

A

TTP, HUS

116
Q

Shiga-like toxin damages

A

capillary endothelial cells

117
Q

triad of HUS

A

thrombocytopenia
MAHA
acute kidney injury

118
Q

TTP is commonly seen in

A

young women

119
Q

autoantibodies against ADAMTS13

A

TTP

120
Q

ADAMSTS13 cleaves

A

large vWF complexes

121
Q

neurologic symptoms caused by microvascular injury

A

TTP

122
Q

splenomegaly/portal hypertension can increase

A

sequestration of platelets in spleen, causing thrombocytopenia

IBI Test 3 (20 decks)

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