CHAPTER 1 - HEMOSTASIS PART 5 Flashcards
(129 cards)
1
Q
BLEEDING DISORDERS
A
- Superficial bleeding
- Deep tissue bleeding
2
Q
Superficial bleeding -Ex:
A
epistaxis, petechiae, gingival bleeding
3
Q
associated with platelet disorder or defect (either in function or in morphology) and with a vascular disorder
A
Superficial bleeding
4
Q
caused by a defect in primary hemostasis
A
Superficial bleeding
5
Q
Deep tissue bleeding -Ex.
A
hemarthrosis, hematomas
6
Q
caused by a defect in secondary hemostasis (clotting or coagulation factors)
A
Deep tissue bleeding
7
Q
QUALITATIVE PLÄTELET DISORDERS Manifested by
A
excessive bruising, superficial bleeding and prolonged BT.
8
Q
External factors: acquired from a viral infection from an inflammation caused by lifestyle or others
A
- Acquired
9
Q
Mild
A
- Acquired
10
Q
Hereditary or inborn errors
A
- Congenital
11
Q
characterized by 3 major platelet function (adhesion, aggregation, platelet/granule release)
A
- Congenital
12
Q
- problem or defect with the interaction of platelet and blood vessel
A
Adhesion Defects (Platelet-vessel wall interaction)
13
Q
BernardSoulier Syndrome
• Deficiency in
A
Gp Ib/IX complex
14
Q
- complex responsible for adhesion
A
Gp Ib/IX and von Willebrand complex
15
Q
BernardSoulier Syndrome
• Characterics:
A
large platelets and thrombocytopenia
16
Q
BernardSoulier Syndrome
• Laboratory:
A
Prolonged Bleeding Time
17
Q
No platelet count formed because the platelet cannot adhere to the bv/skin
A
BernardSoulier Syndrome
18
Q
• Aggregation studies
a. Normal with:
b. Abnormal with:
A
Epinephrine, ADP, Collagen
Ristocetin
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
20
Q
BernardSoulier Syndrome
Treatment: cannot be corrected by adding [?]; no specific treatment; [?]–
A
normal plasma or cryoprecipitate
Desmosin acetate + recombinant factor Vila
21
Q
used to arrest the bleeding if needed
A
Desmosin acetate + recombinant factor Vila
22
Q
generate clot using tissue factors
A
Desmosin acetate + recombinant factor Vila
23
Q
temporary treatment
A
Desmosin acetate + recombinant factor Vila
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
25
manifested in infancy or childhood
BernardSoulier Syndrome
26
BernardSoulier Syndrome signs and symtoms:
1) epistaxis, 2) gingival bleeding, and 3) ecchymosis
27
4 glycoproteins required to form Gp Ib/IX complex:
o Gp Iba – Chromosome 17
o Gp IbB – Chromosome 22
o Gp IbIX – Chromosome 3
o Gp V – Chromosome 3
28
: attaches thrombin and VWF to the injury site
o Gp Iba – Chromosome 17
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: major complex for adhesion but enhanced with Gp V
Gp Ib/IX
30
enhances the fx of these receptors to complete the adhesion complex (Gp Ib/IX and von Willebrand complex)
Gp V
31
: complex is present, but nonfunctional/defective
Pseudo Bernard-Soulier Syndrome
32
von Willebrand disease
• (Autosomal) deficiency in
plasma VIII:vWF (Factor VIII complex)
33
cannot exist alone like VIII
vWF
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– in complex with a multimerprotein, w/c is vWF
Factor VIII complex
35
• Same aggregation test result with Bernard-Soulier
von Willebrand disease
36
von Willebrand disease
• Common sign/Characterics:
mucocutaneous bleeding
37
von Willebrand disease
• Laboratory:
Platelet count and morphology are generally NORMAL
38
von Willebrand disease
• Aggregation studies:
a. Normal with:
b. Abnormal with:
Epinephrine, ADP, Collagen
Ristocetin
39
von Willebrand disease
• Diagnosis:
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
40
von Willebrand disease
• Treatment:
Cryoprecipitate; Desmopressin acetate (1-desamino-8-D arginine vasopressin/ DDAVP)
41
Cryoprecipitate contains:
F I (fibrinogen), F VIII, F XIII, VWF, Fibronectin
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: adhesive ligand (causes the production of clot
V Fibronectin
43
promotes spreading of plt and adhesion of wbc to avoid infection
Fibronectin
44
: causes the release of VWF antigen in the linings of bv
Desmopressin acetate
45
: carries F VIII complex
VWF antigen
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Partial quantitative deficiency of von Willebrand factor (VWF); approximately 75%
1
47
Qualitative deficiency of VWF
2
48
weight multimers (the WWF is more susceptible to proteolysis by ADAMTS-13)
2A
49
Increased affinity for platelet glycoprotein Ib/IXN
2B
50
Decreased platelet receptor binding
2M
51
(Normandy Variant) Impaired factor VIII binding site
2N
52
WWF is absent or nearly absent from plasma
3
53
Glanzmann's Thrombasthenia
Deficiency in
Gp IIb-IIIa
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(receptor for aggregation)
Gp IIb-IIIa
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Glanzmann's Thrombasthenia
• Characterics:
Platelet tends to remain isolated
56
Defect in the hemostati/platelet plug
Glanzmann's Thrombasthenia
57
Glanzmann's Thrombasthenia
• Laboratory:
a. Normal plt ct and morphology
b. Abnormal in vitro clot reaction
c. Thrombosthenia
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compact plt clot will be reduced and mediated by actomyosin or thrombostenin of the activated plts (helps the clot to undergo fibrinolysis)
Glanzmann's Thrombasthenia
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week plts cannot contract; clot retraction is abnormal
c. Thrombosthenia
60
Glanzmann's Thrombasthenia
• Aggregation studies:
a. Abnormal with:
b. Normal with:
Epinephrine, ADP, Collagen
Ristocetin
61
- is a strong CF to cause plt release even if not aggregated
Thrombin
62
allows plt release during adhesion even w/o aggregation to still help arrest bleeding
Thrombin
63
activated normal protein/plts
Thrombin
64
Glanzmann's Thrombasthenia
• Treatment:
Recombinant Factor VIIa
65
: induces thrombus formation using tissue factors/tissue dependent generators near the lesion to stop bleeding
Factor VIIa
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: complete absence of fibrinogen
- Afibrinogenemia
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: low level of fibrinogen
- Hypofibrinogenemia
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: occurs during infancy and childhood
- Congenital
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- Severe glycoprotein deficiency
(Congenital) Afibrinogenemia or Hypofibrinogenemia
70
(Congenital) Afibrinogenemia or Hypofibrinogenemia
• Laboratory:
a. Normal plt ct
b. Abnormal structure
71
larger platelets (gray to bluegray)
Gray platelet syndrome
72
normal platelet
Quebec Platelet Disorder
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thrombocytopenia (consistent)
Gray platelet syndrome
74
abnormal proteolysis
Quebec Platelet Disorder
75
Gray platelet syndrome Laboratory
a. Prolonged Bleeding time
b. Decreased aggregation with all agents
76
Quebec Platelet Disorder Laboratory
a. Delayed (12-24 hrs) mucocutaneous bleeding
b. Thrombocytopenia (inconsistent)
77
WASp mutation
WiskottAldrich
78
Mutation in Ch 19
HermanskyPudlak
79
Mutation in Ch 13
Chediak Higashi
80
Mutation in RBM8A
TAR Syndrome
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small platelets
WiskottAldrich
82
Swiss cheese platelet
HermanskyPudlak
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fused granules of lysosome
Chediak Higashi
84
normal platelet
TAR Syndrome
85
WiskottAldrich
Triad of Symptoms
Thrombocytopenia
Recurrent infxn
Eczema
86
HermanskyPudlak
Triad of Symptoms
Occulocutaneous albinism
Ceroid-like pigment in macrophage
Bleeding tendency
87
Chediak Higashi
Triad of Symptoms
Albinism
Recurrent infection
Giant lysosomes
88
TAR Syndrome
Triad of Symptoms
Thrombocytopenia
Absence/hypoplasia of radial bones
Abnormal aggregation (congenital)
89
• Deficiency of alpha granules
Gray platelet syndrome
90
Gray platelet syndrome
• Characterized by
a) larger platelets colored gray to blue-gray, and a) thrombocytopenia
91
Gray platelet syndrome
• Laboratory:
a. Prolonged Bleeding time
b. Decreased aggregation with all agents
92
• Autosomal dominant disorder characterized by abnormal proteolysis & deficiency of alpha granules
Quebec Platelet Disorder
93
Quebec Platelet Disorder
• Characterized by
a) normal platelet, and b) abnormal proteolysis
94
Enzymes are abnormal because they target the alpha granules and degrades it even if not in use
Quebec Platelet Disorder
95
Alpha granules are stimulated to release content, but here, it is degraded and proeteolysed
Quebec Platelet Disorder
96
• Characterized by delayed (12-24 hrs) mucocutaneous bleeding. - Not onset
Quebec Platelet Disorder
97
Quebec Platelet Disorder
• Laboratory:
a. Thrombocytoenia (inconsistent)
98
(Sex-linked) deficiency dense granules, with a defect in cytoskeletal assembly.
Wiskott-Aldrich syndrome
99
Characterized by predominance of small platelets
Wiskott-Aldrich syndrome
100
• Triad of Symptoms include
- thrombocytopenia, recurrent infection & eczema
Wiskott-Aldrich syndrome
101
Remodels cytoskeleton
Wiskott-Aldrich Protein (WASp)
102
Assembly of cytoskeleton becomes defective
Wiskott-Aldrich Protein (WASp)
103
Wiskott-Aldrich Protein (WASp) Plt cannot activate not conrtact resulting to impaired
1) migration, 2) adhesion, and 1) interaction
104
Deficiency of dense granules
HermanskyPudlak syndrome
Chediak-Higashi
105
Triad of symptom include oculocutaneous albinism, accumulation of ceroid-like pigment in macrophages & bleeding tendencies
HermanskyPudlak syndrome
106
Plt has marked dilation (enlargement of surface connecting system and dense tubular system resulting to cheese appearance
Swiss cheese platelet
107
First found when rats were injured when injected with lead acetate
Swiss cheese platelet
108
4 divisions of granules in the lysosome
Chediak-Higashi
109
Causes a generalized cell dysfunction
Chediak-Higashi
110
Cytoplasmic granule fusion - lysosome in the cytoplasm
Chediak-Higashi
111
May lead to lymphocytic proliferation in the liver cells, spleen, and marrow
Chediak-Higashi
112
Macrophage accumulates in the tissues
Chediak-Higashi
113
Pancytopenia: most dangerous; decrease in all blood cells
Chediak-Higashi
114
• Structural defect in beta granules with corresponding abnormal aggregation responses and thrombocytopenia
Thrombocytopenia with Absent Radii syndrome (TAR)
115
• Characterized by severe neonatal thrombocytopenia and congenital absence or extreme hypoplasia of the radial bones (most pronounced skeletal abnormality), cardiac and other skeletal abnormalities.
Thrombocytopenia with Absent Radii syndrome (TAR)
116
Very low platelet count = shortens plt production
Thrombocytopenia with Absent Radii syndrome (TAR)
117
: inside
phosphatidylserine (PS) & phosphatidylethanolamine (PE)
118
: outside
phosphatidylcholine
119
happens during activation of platelet
Flip
120
caused by an enzyme called scramblase
Flip
121
phosphatidylserine switches with phosphatidylcholine
Flip
122
phosphatidylserine and phosphatidylethanolamine in the outer leaflet facilitates the assembly of clotting factors in normal condition
Flip
123
Surface expression of phosphatidylserine is decreased
Scott syndrome
124
skipping the plt to be activated
Scott syndrome
125
Platelets are always in an activated state without prior activation.
Stormorken syndrome
126
keeps the plt inactivated
Stormorken syndrome
127
flip is normal
Stormorken syndrome
128
Defective amino phospholipid translocase
Stormorken syndrome
129
: responsible for bringing phosphatidylserine to go back inside during resting platelet (not inactivated)
translocase
