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Hematology 2 Lec > CHAPTER 1 - HEMOSTASIS PART 5 > Flashcards

CHAPTER 1 - HEMOSTASIS PART 5 Flashcards

1
Q

BLEEDING DISORDERS

A
  1. Superficial bleeding
  2. Deep tissue bleeding
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2
Q

Superficial bleeding -Ex:

A

epistaxis, petechiae, gingival bleeding

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3
Q

associated with platelet disorder or defect (either in function or in morphology) and with a vascular disorder

A

Superficial bleeding

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4
Q

caused by a defect in primary hemostasis

A

Superficial bleeding

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5
Q

Deep tissue bleeding -Ex.

A

hemarthrosis, hematomas

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6
Q

caused by a defect in secondary hemostasis (clotting or coagulation factors)

A

Deep tissue bleeding

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7
Q

QUALITATIVE PLÄTELET DISORDERS Manifested by

A

excessive bruising, superficial bleeding and prolonged BT.

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8
Q

External factors: acquired from a viral infection from an inflammation caused by lifestyle or others

A
  1. Acquired
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9
Q

Mild

A
  1. Acquired
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10
Q

Hereditary or inborn errors

A
  1. Congenital
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11
Q

characterized by 3 major platelet function (adhesion, aggregation, platelet/granule release)

A
  1. Congenital
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12
Q
  • problem or defect with the interaction of platelet and blood vessel
A

Adhesion Defects (Platelet-vessel wall interaction)

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13
Q

BernardSoulier Syndrome
• Deficiency in

A

Gp Ib/IX complex

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14
Q
  • complex responsible for adhesion
A

Gp Ib/IX and von Willebrand complex

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15
Q

BernardSoulier Syndrome
• Characterics:

A

large platelets and thrombocytopenia

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16
Q

BernardSoulier Syndrome
• Laboratory:

A

Prolonged Bleeding Time

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17
Q

No platelet count formed because the platelet cannot adhere to the bv/skin

A

BernardSoulier Syndrome

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18
Q

• Aggregation studies

a. Normal with:
b. Abnormal with:

A

Epinephrine, ADP, Collagen

Ristocetin

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19
Q

(in-vitro; collection of blood sample and induce aggregation using the ff. chemicals that are also found in the body that stimulates platelet aggregation and adhesion):

A

• Aggregation studies

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20
Q

BernardSoulier Syndrome
Treatment: cannot be corrected by adding [?]; no specific treatment; [?]–

A

normal plasma or cryoprecipitate

Desmosin acetate + recombinant factor Vila

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21
Q

used to arrest the bleeding if needed

A

Desmosin acetate + recombinant factor Vila

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22
Q

generate clot using tissue factors

A

Desmosin acetate + recombinant factor Vila

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23
Q

temporary treatment

A

Desmosin acetate + recombinant factor Vila

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24
Q

Transfusion of normal plasma will still continue bleeding; Platelet transfused will be worn out; Original plt will still have the defect of adhesion

A

BernardSoulier Syndrome

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25
Q

manifested in infancy or childhood

A

BernardSoulier Syndrome

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26
Q

BernardSoulier Syndrome signs and symtoms:

A

1) epistaxis, 2) gingival bleeding, and 3) ecchymosis

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27
Q

4 glycoproteins required to form Gp Ib/IX complex:

A

o Gp Iba – Chromosome 17
o Gp IbB – Chromosome 22
o Gp IbIX – Chromosome 3
o Gp V – Chromosome 3

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28
Q

: attaches thrombin and VWF to the injury site

A

o Gp Iba – Chromosome 17

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29
Q

: major complex for adhesion but enhanced with Gp V

A

Gp Ib/IX

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30
Q

enhances the fx of these receptors to complete the adhesion complex (Gp Ib/IX and von Willebrand complex)

A

Gp V

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31
Q

: complex is present, but nonfunctional/defective

A

Pseudo Bernard-Soulier Syndrome

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32
Q

von Willebrand disease
• (Autosomal) deficiency in

A

plasma VIII:vWF (Factor VIII complex)

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33
Q

cannot exist alone like VIII

A

vWF

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34
Q

– in complex with a multimerprotein, w/c is vWF

A

Factor VIII complex

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35
Q

• Same aggregation test result with Bernard-Soulier

A

von Willebrand disease

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36
Q

von Willebrand disease
• Common sign/Characterics:

A

mucocutaneous bleeding

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37
Q

von Willebrand disease
• Laboratory:

A

Platelet count and morphology are generally NORMAL

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38
Q

von Willebrand disease
• Aggregation studies:

a. Normal with:
b. Abnormal with:

A

Epinephrine, ADP, Collagen

Ristocetin

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39
Q

von Willebrand disease
• Diagnosis:

A

Standard WWD test panel tests:
1. quantitative VWF test (VWF: Ag assay)
2. VWF activity test/ VWF:RCo assay (qualitative; ability of WWF to bind to platelets)
3. factor VIll activity assay

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40
Q

von Willebrand disease
• Treatment:

A

Cryoprecipitate; Desmopressin acetate (1-desamino-8-D arginine vasopressin/ DDAVP)

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41
Q

Cryoprecipitate contains:

A

F I (fibrinogen), F VIII, F XIII, VWF, Fibronectin

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42
Q

: adhesive ligand (causes the production of clot

A

V Fibronectin

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43
Q

promotes spreading of plt and adhesion of wbc to avoid infection

A

Fibronectin

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44
Q

: causes the release of VWF antigen in the linings of bv

A

Desmopressin acetate

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45
Q

: carries F VIII complex

A

VWF antigen

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46
Q

Partial quantitative deficiency of von Willebrand factor (VWF); approximately 75%

A

1

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47
Q

Qualitative deficiency of VWF

A

2

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48
Q

weight multimers (the WWF is more susceptible to proteolysis by ADAMTS-13)

A

2A

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49
Q

Increased affinity for platelet glycoprotein Ib/IXN

A

2B

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50
Q

Decreased platelet receptor binding

A

2M

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51
Q

(Normandy Variant) Impaired factor VIII binding site

A

2N

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52
Q

WWF is absent or nearly absent from plasma

53
Q

Glanzmann’s Thrombasthenia
Deficiency in

A

Gp IIb-IIIa

54
Q

(receptor for aggregation)

A

Gp IIb-IIIa

55
Q

Glanzmann’s Thrombasthenia
• Characterics:

A

Platelet tends to remain isolated

56
Q

Defect in the hemostati/platelet plug

A

Glanzmann’s Thrombasthenia

57
Q

Glanzmann’s Thrombasthenia
• Laboratory:

A

a. Normal plt ct and morphology
b. Abnormal in vitro clot reaction
c. Thrombosthenia

58
Q

compact plt clot will be reduced and mediated by actomyosin or thrombostenin of the activated plts (helps the clot to undergo fibrinolysis)

A

Glanzmann’s Thrombasthenia

59
Q

week plts cannot contract; clot retraction is abnormal

A

c. Thrombosthenia

60
Q

Glanzmann’s Thrombasthenia
• Aggregation studies:
a. Abnormal with:
b. Normal with:

A

Epinephrine, ADP, Collagen

Ristocetin

61
Q
  • is a strong CF to cause plt release even if not aggregated
62
Q

allows plt release during adhesion even w/o aggregation to still help arrest bleeding

63
Q

activated normal protein/plts

64
Q

Glanzmann’s Thrombasthenia
• Treatment:

A

Recombinant Factor VIIa

65
Q

: induces thrombus formation using tissue factors/tissue dependent generators near the lesion to stop bleeding

A

Factor VIIa

66
Q

: complete absence of fibrinogen

A
  • Afibrinogenemia
67
Q

: low level of fibrinogen

A
  • Hypofibrinogenemia
68
Q

: occurs during infancy and childhood

A
  • Congenital
69
Q
  • Severe glycoprotein deficiency
A

(Congenital) Afibrinogenemia or Hypofibrinogenemia

70
Q

(Congenital) Afibrinogenemia or Hypofibrinogenemia
• Laboratory:

A

a. Normal plt ct
b. Abnormal structure

71
Q

larger platelets (gray to bluegray)

A

Gray platelet syndrome

72
Q

normal platelet

A

Quebec Platelet Disorder

73
Q

thrombocytopenia (consistent)

A

Gray platelet syndrome

74
Q

abnormal proteolysis

A

Quebec Platelet Disorder

75
Q

Gray platelet syndrome Laboratory

A

a. Prolonged Bleeding time
b. Decreased aggregation with all agents

76
Q

Quebec Platelet Disorder Laboratory

A

a. Delayed (12-24 hrs) mucocutaneous bleeding
b. Thrombocytopenia (inconsistent)

77
Q

WASp mutation

A

WiskottAldrich

78
Q

Mutation in Ch 19

A

HermanskyPudlak

79
Q

Mutation in Ch 13

A

Chediak Higashi

80
Q

Mutation in RBM8A

A

TAR Syndrome

81
Q

small platelets

A

WiskottAldrich

82
Q

Swiss cheese platelet

A

HermanskyPudlak

83
Q

fused granules of lysosome

A

Chediak Higashi

84
Q

normal platelet

A

TAR Syndrome

85
Q

WiskottAldrich
Triad of Symptoms

A

Thrombocytopenia
Recurrent infxn
Eczema

86
Q

HermanskyPudlak
Triad of Symptoms

A

Occulocutaneous albinism
Ceroid-like pigment in macrophage
Bleeding tendency

87
Q

Chediak Higashi
Triad of Symptoms

A

Albinism
Recurrent infection
Giant lysosomes

88
Q

TAR Syndrome
Triad of Symptoms

A

Thrombocytopenia
Absence/hypoplasia of radial bones
Abnormal aggregation (congenital)

89
Q

• Deficiency of alpha granules

A

Gray platelet syndrome

90
Q

Gray platelet syndrome
• Characterized by

A

a) larger platelets colored gray to blue-gray, and a) thrombocytopenia

91
Q

Gray platelet syndrome
• Laboratory:

A

a. Prolonged Bleeding time
b. Decreased aggregation with all agents

92
Q

• Autosomal dominant disorder characterized by abnormal proteolysis & deficiency of alpha granules

A

Quebec Platelet Disorder

93
Q

Quebec Platelet Disorder
• Characterized by

A

a) normal platelet, and b) abnormal proteolysis

94
Q

Enzymes are abnormal because they target the alpha granules and degrades it even if not in use

A

Quebec Platelet Disorder

95
Q

Alpha granules are stimulated to release content, but here, it is degraded and proeteolysed

A

Quebec Platelet Disorder

96
Q

• Characterized by delayed (12-24 hrs) mucocutaneous bleeding. - Not onset

A

Quebec Platelet Disorder

97
Q

Quebec Platelet Disorder
• Laboratory:

A

a. Thrombocytoenia (inconsistent)

98
Q

(Sex-linked) deficiency dense granules, with a defect in cytoskeletal assembly.

A

Wiskott-Aldrich syndrome

99
Q

Characterized by predominance of small platelets

A

Wiskott-Aldrich syndrome

100
Q

• Triad of Symptoms include
- thrombocytopenia, recurrent infection & eczema

A

Wiskott-Aldrich syndrome

101
Q

Remodels cytoskeleton

A

Wiskott-Aldrich Protein (WASp)

102
Q

Assembly of cytoskeleton becomes defective

A

Wiskott-Aldrich Protein (WASp)

103
Q

Wiskott-Aldrich Protein (WASp) Plt cannot activate not conrtact resulting to impaired

A

1) migration, 2) adhesion, and 1) interaction

104
Q

Deficiency of dense granules

A

HermanskyPudlak syndrome

Chediak-Higashi

105
Q

Triad of symptom include oculocutaneous albinism, accumulation of ceroid-like pigment in macrophages & bleeding tendencies

A

HermanskyPudlak syndrome

106
Q

Plt has marked dilation (enlargement of surface connecting system and dense tubular system resulting to cheese appearance

A

Swiss cheese platelet

107
Q

First found when rats were injured when injected with lead acetate

A

Swiss cheese platelet

108
Q

4 divisions of granules in the lysosome

A

Chediak-Higashi

109
Q

Causes a generalized cell dysfunction

A

Chediak-Higashi

110
Q

Cytoplasmic granule fusion - lysosome in the cytoplasm

A

Chediak-Higashi

111
Q

May lead to lymphocytic proliferation in the liver cells, spleen, and marrow

A

Chediak-Higashi

112
Q

Macrophage accumulates in the tissues

A

Chediak-Higashi

113
Q

Pancytopenia: most dangerous; decrease in all blood cells

A

Chediak-Higashi

114
Q

• Structural defect in beta granules with corresponding abnormal aggregation responses and thrombocytopenia

A

Thrombocytopenia with Absent Radii syndrome (TAR)

115
Q

• Characterized by severe neonatal thrombocytopenia and congenital absence or extreme hypoplasia of the radial bones (most pronounced skeletal abnormality), cardiac and other skeletal abnormalities.

A

Thrombocytopenia with Absent Radii syndrome (TAR)

116
Q

Very low platelet count = shortens plt production

A

Thrombocytopenia with Absent Radii syndrome (TAR)

117
Q

: inside

A

phosphatidylserine (PS) & phosphatidylethanolamine (PE)

118
Q

: outside

A

phosphatidylcholine

119
Q

happens during activation of platelet

120
Q

caused by an enzyme called scramblase

121
Q

phosphatidylserine switches with phosphatidylcholine

122
Q

phosphatidylserine and phosphatidylethanolamine in the outer leaflet facilitates the assembly of clotting factors in normal condition

123
Q

Surface expression of phosphatidylserine is decreased

A

Scott syndrome

124
Q

skipping the plt to be activated

A

Scott syndrome

125
Q

Platelets are always in an activated state without prior activation.

A

Stormorken syndrome

126
Q

keeps the plt inactivated

A

Stormorken syndrome

127
Q

flip is normal

A

Stormorken syndrome

128
Q

Defective amino phospholipid translocase

A

Stormorken syndrome

129
Q

: responsible for bringing phosphatidylserine to go back inside during resting platelet (not inactivated)

A

translocase

Hematology 2 Lec (29 decks)

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