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HEMATOLOGY2 > MIDTERM > Flashcards

MIDTERM Flashcards

1
Q

manifests in infancy or childhood with hemorrhage characteristics of defective platelet function: ecchymoses, epistaxis, and gingival bleeding

A

Bernard-Soulier (Giant Platelet) Syndrome

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2
Q

problem: lack or abnormal GP Ib/IX/V complex in platelet surface

A

Bernard-Soulier (Giant Platelet) Syndrome

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3
Q

resembles VWD

A

Bernard-Soulier (Giant Platelet) Syndrome

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4
Q

homozygotes for BSS

A

50%

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5
Q

heterozygotes for BSS

A

enlarged platelets, thrombocytopenia, and usually decreased platelet survival

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6
Q

BSS most common form

A

defect in GP Ib⍺

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7
Q

Bernard-Soulier (Giant Platelet) Syndrome (laboratory profile )

A

normal platelet aggregation in response to platelet activating agents binding of VWF to platelets
abnormal platelet aggregation: ristocetin-induced

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8
Q

large platelets and thrombocytopenia

A

Inherited Giant Platelet Syndromes

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9
Q

Inherited Giant Platelet Syndromes platelet ultrastructure:

A

normal

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10
Q

abnormal microtubule distribution

A

May-Hegglin anomaly

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11
Q

platelets are spherical and have a prominent surface-connected canalicular system.

A

Epstein syndrome

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12
Q

Hermansky-Pudlak syndrome
Chédiak-Higashi syndrome problem

A

problem: platelet dense granule deficiency

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13
Q

tyrosinase- positive oculocutaneous albinism

A

Hermansky-Pudlak syndrome

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14
Q

defective lysosomal function

A

Hermansky-Pudlak syndrome

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15
Q

ceroid-like deposition in the cells of the RES

A

Hermansky-Pudlak syndrome

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16
Q

morphologic abnormality: marked dilation and tortuosity of SCCS

A

Hermansky-Pudlak syndrome

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17
Q

partal oculocutaneous albinism

A

Chédiak-Higashi syndrome

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18
Q

frequent pyogenic bacterial infectons

A

Chédiak-Higashi syndrome

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19
Q

giant lysosomal granules in cells

A

Chédiak-Higashi syndrome

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20
Q

“accelerated: lymphocytic proliferaton in the liver, spleen, and marrow with macrophage accumulation in tissues>pancytopenia>hemorrhage &
increased susceptibility to infecton”

A

Chédiak-Higashi syndrome

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21
Q

problem: absence of Wiskott-Aldrich syndrome protein

A

Wiskott-Aldrich syndrome (WAS)

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22
Q

“problem: absence of Wiskott-Aldrich syndrome protein
>impaired migration>impaired adhesion”

A

Wiskott-Aldrich syndrome (WAS)

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23
Q

classic form: thrombocytopenia immunodeficiency syndrome

A

Wiskott-Aldrich syndrome (WAS)

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24
Q

Wiskott-Aldrich syndrome (WAS) classic form:

A

thrombocytopenia immunodeficiency syndrome

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25
Q

thrombocytopenia immunodeficiency syndrome (WAS classic form) association

A

susceptibility to infectons
microthrombocytopenia
severe eczema

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26
Q

platelets: abnormal structure and decreased dense granules

A

Wiskott-Aldrich syndrome (WAS)

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27
Q

presence of microthrombocytes: TORCH

A

Wiskott-Aldrich syndrome (WAS)

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28
Q

Wiskott-Aldrich syndrome (WAS) (Laboratory profile)

A

decreased aggregation response (ADP, collagen, and epinephrine)

normal: aggregtion response (thrombin) (Laboratory profile)

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29
Q

problem: radial bones (most pronounced skeletal abnormality)

A

Thrombocytopenia with absent radii syndrome (TAR)

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30
Q

platelets have structural defects in dense granules

A

Thrombocytopenia with absent radii syndrome (TAR)

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31
Q

abnormal aggregation responses

A

Thrombocytopenia with absent radii syndrome (TAR)

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32
Q

megakaryocytes: decreased, immature, or normal

A

Thrombocytopenia with absent radii syndrome (TAR)

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33
Q

problem: absence of morphologically recognizable ⍺-granules in platelets

A

Gray platelet syndrome

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34
Q

lifelong mild bleeding tendencies

A

Gray platelet syndrome

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35
Q

moderate thrombocytopenia

A

Gray platelet syndrome

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36
Q

fibrosis of the marrow

A

Gray platelet syndrome

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37
Q

large platelets whose gray appearance

A

Gray platelet syndrome

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38
Q

large platelets whose gray appearance

A

Gray platelet syndrome

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39
Q

decreased plasma PF4 and β-thromboglobulin (laboratory profile)

A

Gray platelet syndrome

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40
Q

problem: deficiency in multimerin

A

Quebec platelet disorder

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41
Q

protein that is stored complexed with factor V in ⍺-granules

A

multimerin

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42
Q

inhibition of cyclooxygenase: aspirin & ibuprofen > TXA2

A

Thromboxane Pathway Disorders: Aspirin-Like Effects

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43
Q

decreased: aggregation response

A

Thromboxane Pathway Disorders: Aspirin-Like Effects

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44
Q

normal: ultrastructure and granular contents

A

Thromboxane Pathway Disorders: Aspirin-Like Effects

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45
Q

deficiency of ⍺2β1 (GP Ia/IIa) integrin

A

Collagen Receptors

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46
Q

deficiency of GP VI

A

Collagen Receptors

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47
Q

abnormal aggregation response to collagen

A

deficiency of ⍺2β1 (GP Ia/IIa) integrin

47
Q

abnormal aggregation response to collagen

A

deficiency of ⍺2β1 (GP Ia/IIa) integrin

48
Q

platelets did not adhere to collagen

A

deficiency of ⍺2β1 (GP Ia/IIa) integrin

49
Q

lifelong mild bleeding disorder

A

deficiency of ⍺2β1 (GP Ia/IIa) integrin

50
Q

mild bleeding

A

deficiency of GP VI

51
Q

abnormal aggregation and adhesion response to collagen

A

deficiency of GP VI

52
Q

associated with gray platelet syndrome

A

deficiency of GP VI

53
Q

“mediate calcium mobilizaton and shape change in response
to ADP”

54
Q

“macroscopic platelet aggrega;on and is coupled to adenylate
cyclase through a G-inhibitory (Gi) protein complex”

55
Q

decreased platelet aggrega;on in response to ADP

56
Q

normal platelet shape change and calcium mobiliza;on

57
Q

disorder of calcium-induced membrane phospholipid scrambling

A

Scott Syndrome

58
Q

platelets are always in an “activated”

A

Stormorken Syndrome

59
Q

phosphatidylserine on the outer leaflet of the membrane without prior activation

A

Stormorken Syndrome

60
Q

“associated with abnormal in vitro clot retraction and a
normal platelet count”

A

Glanzmann Thrombasthenia (GT)

61
Q

“petechiae, purpura, menorrhagia, gastrointestinal
bleeding, and hematuria”

A

Glanzmann Thrombasthenia (GT)

62
Q

problem: deficiency or abnormality of the platelet membrane GP IIb/IIIa (⍺IIbβ3)

A

Glanzmann Thrombasthenia (GT)

63
Q

homozygotes: deficient > serious bleeding manifestations

A

Glanzmann Thrombasthenia (GT)

64
Q

“principle: pro-⍺Iib>⍺IIb (megakaryocytes) complexed with β3 (ER) > ⍺IIbβ3 complex (Golgi body)”

A

Glanzmann Thrombasthenia (GT)

65
Q

acquired (thrombasthenia-like state)

A

Glanzmann Thrombasthenia (GT)

66
Q

development of autoan;bodies against GP IIb/IIIa

A

acquired (thrombasthenia-like state) (GT)

67
Q

multiple myeloma in which the paraprotein is directed against GP IIIa

A

acquired (thrombasthenia-like state) (GT)

68
Q

afibrinogenemia

A

acquired (thrombasthenia-like state) (GT)

69
Q

Glanzmann Thrombasthenia (GT) (laboratory profile)

A

normal platelet count
normal platelet morphology

70
Q

lack platelet aggregation in response to all platelet activating agents

A

Glanzmann Thrombasthenia (GT) (laboratory profile)

71
Q

induced release/secretion reaction by stimulation with strong agonists

A

Glanzmann Thrombasthenia (GT) (laboratory profile)

72
Q

normal platelet aggregation: ristocetin-induced binding of VWF to platelets

A

Glanzmann Thrombasthenia (GT) (laboratory profile)

72
Q

normal platelet aggregation: ristocetin-induced binding of VWF to platelets

A

Glanzmann Thrombasthenia (GT) (laboratory profile)

73
Q

markedly fewer microvesicles are produced

A

Decreased platelet procoagulant activity (PF 3 test)

74
Q

prothrombin binds directly to GP IIb/IIIa

A

Decreased platelet procoagulant activity (PF 3 test)

75
Q

Collagen Receptors

A

Decreased platelet procoagulant activity (PF 3 test)

76
Q

normal laboratory profile of platelets and blood coagulation

A

Vascular Disorders

77
Q

diagnosis is based on medical history and is made by ruling out other sources of bleeding disorders

A

Vascular Disorders

78
Q

clinical sign: tendency to bruise easily or to bleed spontaneously (mucosal surfaces)

A

Vascular Disorders

79
Q

thin-walled blood vessels with a discontinuous endothelium

A

“Hereditary Hemorrhagic Telangiectasia (Rendu-Osler-Weber Syndrome)”

80
Q

inadequate smooth muscle

A

“Hereditary Hemorrhagic Telangiectasia (Rendu-Osler-Weber Syndrome)”

81
Q

inadequate or missing elastin in the surrounding stroma

A

“Hereditary Hemorrhagic Telangiectasia (Rendu-Osler-Weber Syndrome)”

82
Q

occur throughout the body (face, lips, tongue, conjunc;va, nasal mucosa, fingers, toes, and trunk and under the tongue)

A

“Hereditary Hemorrhagic Telangiectasia (Rendu-Osler-Weber Syndrome)”

83
Q

lesions blanch when pressure is applied

A

“Hereditary Hemorrhagic Telangiectasia (Rendu-Osler-Weber Syndrome)”

84
Q

usually manifests by puberty and progresses throughout life.

A

“Hereditary Hemorrhagic Telangiectasia (Rendu-Osler-Weber Syndrome)”

85
Q

cherry-red hemangiomas: (common in older men and women

A

“Hereditary Hemorrhagic Telangiectasia (Rendu-Osler-Weber Syndrome)”

86
Q

ataxia-telangiectasia: Louis-Bar syndrome

A

“Hereditary Hemorrhagic Telangiectasia (Rendu-Osler-Weber Syndrome)”

87
Q

chronic liver disease
pregnancy

A

“Hereditary Hemorrhagic Telangiectasia (Rendu-Osler-Weber Syndrome)”

88
Q

giant cavernous hemangioma

A

“Hemangioma-Thrombocytopenia Syndrome (Kasabach- Merritt
Syndrome)

89
Q

thrombocytopenia
bleeding diathesis

A

“Hemangioma-Thrombocytopenia Syndrome (Kasabach- Merritt
Syndrome)

90
Q

complications:
acute or chronic disseminated intravascular coagulation

sequestration of platelets in the hemangiomas

microangiopathic hemolytic anemia

A

“Hemangioma-Thrombocytopenia Syndrome (Kasabach- Merritt
Syndrome)

91
Q

defects in collagen production, structure, or crosslinking, with resulting inadequacy of the connective tissues

A

Ehlers-Danlos Syndrome

92
Q

hyperextensible skin

A

Ehlers-Danlos Syndrome

93
Q

hypermobile joints
joint laxity
fragile tissues
bleeding tendency (subcutaneous hematoma formation)

A

Ehlers-Danlos Syndrome

94
Q

allergic/ anaphylactoid purpura
allergic manifesta;ons (skin rash and edema)
associated with foods and drugs, cold, insect bites, and vaccinations

A

Henoch-Schönlein Purpura Allergic Purpura

95
Q

acute IgA-mediated disorder with widespread generalized vasculitis involving the skin, joints, kidneys, gastrointestinal tract, and, less commonly, the lungs.

A

Henoch-Schönlein Purpura Allergic Purpura

96
Q

purpuric skin lesions are frequently confused with the hemorrhagic rash of immune thrombocytopenic purpura
disease of children (3-7 years of age); more common in males
“palpable purpura’, itchy purpuric lesions

A

Henoch-Schönlein Purpura Allergic Purpura

97
Q

(+) proteinuria and hematuria

A

Henoch-Schönlein Purpura Allergic Purpura

98
Q

normal: platelet count & blood coagulation tests

A

Henoch-Schönlein Purpura Allergic Purpura

99
Q

increased: WBC & ESR

A

Henoch-Schönlein Purpura Allergic Purpura

100
Q

platelet func;on can be inhibited by myeloma proteins

A

Paraproteinemia

101
Q

coating of the platelet membrane with the paraprotein >abnormalities in platelet aggregation, secretion, and procoagulant activity

A

Paraproteinemia

102
Q

exhibit platelet function abnormalities

A

IgA myeloma, Waldenström macroglobulinemia & IgG3 myeloma

103
Q

inhibiting fibrin polymerization

A

“poor correlation between abnormal results on laboratory tests and evidence of clinical bleeding.”

104
Q

deposition of abnormal quantities of amyloid protein in tissues

A

Amyloidosis

105
Q

fibrous protein with rigid, linear, non- branching, aggregated fibrils

106
Q

primary or secondary, and localized or systemic

A

Amyloidosis

107
Q

characterized by: purpura, hemorrhage, and thrombosis

A

Amyloidosis

108
Q

abnormal platelet function

A

Amyloidosis

109
Q

occurs more commonly in elderly men than in women

A

Senile Purpura

110
Q

“due to a lack of collagen support for small blood vessels and loss of subcutaneous fat and elastic fibers”

A

Senile Purpura

111
Q

“dark blotches are flattened, do not blanch with pressure, and resolve slowly,
often leaving a brown stain in the skin.”

A

Senile Purpura

112
Q

laboratory results: normal
increased: capillary fragility
no other bleeding manifesta;ons

A

Senile Purpura

113
Q

aspirin, warfarin, barbiturates, diuretics, digoxin, methyldopa, sulfonamides and iodides

A

Drug induced

HEMATOLOGY2 (1 decks)

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