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Coagulation > Disorders of Secondary Hemostasis > Flashcards

Disorders of Secondary Hemostasis Flashcards

1
Q

Types of Disorders

A

Platelets
Vessels
Coagulation Factors

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2
Q

Patient Evaluation

A

History & Physical - very important to know
Type of bleeding
Medications
Age
Sex
Family History

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3
Q

Screening Laboratory Tests

A

PT, APTT
TT
Fibrinogen Assay
Platelet Count

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4
Q

Followup Laboratory Tests

A

Bleeding Time
Platelet Function
Vessel Function
Correction Studies
Inhibitor Testing
Factor Assays

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5
Q

Congential Disorders

A

Decreased synthesis of factors
Abnormal function of factors

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6
Q

Aquired Disorders

A

Vitamin K deficiency
DIC
Liver Disease

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7
Q

Factor 1 Inheritance

A

AR Inherited

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8
Q

Factor 1 Clinical Presentation

A

Bleeding
Hemarthrosis

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9
Q

Factor 1 Testing

A

PT - prolonged
APTT - prolonged
BT - prolonged
TT - prolonged
Fibrinogen concentration - decreased

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10
Q

Factor 1 Therapy

A

FFP
Cryoprecipitate
Factor 1 concentrates

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11
Q

Dysfibrinogenemia Inheritance

A

AD Inheritance

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12
Q

Dysfibrinogenemia Clinical Presentation

A

Usually asymptomatic
Bleeding
Thrombosis
Poor Wound Healing

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13
Q

Dysfibrinogenemia Laboratory Testing

A

PT - prolonged
APTT - prolonged
TT - prolonged
RT - prolonged
Fibrinogen concentration - normal
BT - normal

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14
Q

Dysfibrinogenemia Therapy

A

Anticoagulant therapy

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15
Q

Factor 2 Inheritance

A

AR Inheritance

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16
Q

Factor 2 Clinical Presentation

A

Bleeding

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17
Q

Factor 2 Laboratory Testing

A

PT - prolonged
APTT - prolonged
Fibrinogen concentration - normal
BT - normal
TT - normal

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18
Q

Factor 2 Therapy

A

FFP
Prothrombin Complex Concentrate (2, 7, 9, 10)

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19
Q

Factor 5 Inheritance

A

AR Inheritance

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20
Q

Factor 5 Clinical Presentation

A

Bleeding
Bruising

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21
Q

Factor 5 Testing

A

PT - prolonged
APTT - prolonged
Fibrinogen concentration - normal
BT - normal
TT - normal

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22
Q

Factor 5 Therapy

A

FFP
Poss Factor 5 concentration

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23
Q

Factor 5 Leiden Inheritance
Activated Protein C Resistance (APCR)

A

Genetic Mutation
Point where Protein C binds to inactivate Factor 5

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24
Q

Factor 5 Leiden Clinical Presentation

A

Thrombosis

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25
Factor 5 Leiden Laboratory Testing
Modified APTT PCR
26
Factor 5 Leiden Therapy
Anticoagulant Therapy
27
Factor 7 Inheritance
AR Inheritance
28
Factor 7 Clinical Presentation
Bleeding
29
Factor 7 Laboratory Testing
PT - prolonged APTT - normal BT - normal TT - normal Fibrinogen - normal
30
Factor 7 Therapy
FFP Prothrombin complex concentrate
31
Factor 10 Inheritance
AR Inheritance May be acquired with amyloidosis (vessels and organs)
32
Factor 10 Laboratory Testing
PT - prolonged APTT - prolonged BT - normal TT - normal Fibrinogen - normal
33
Factor 10 Therapy
FFP Prothrombin complex concentrate
34
Factor 8 Inheritance Hemophilia A
Sex-linked Recessive - women are carriers
35
Factor 8:c Clinical Presentation
Bleeding - Severe (0-1%), Moderate (2-5%), Mild (5-20%)
36
Factor 8:c Laboratory Testing
APTT - prolonged PT - normal BT - normal TT - normal Fibrinogen - normal 8:c - decreased VWF:Ag - normal or increased VWF:Rco - normal or increased
37
Factor 8:c Therapy
Factor 8 concentrates Counseling
38
Factor 8:c Considerations
Inhibitors - Time and Temperature dependent - Bethesda Titer - No response to therapy - Alternative therapies
39
VWF Inheritance
AD Inheritance
40
VWF Types of disease
Type 1 - Normal structure, defective release Type 2 - Abnormal structure Type 3 - Absent synthesis
41
VWF Clinical Presentation
Bleeding (mucosal) VWF adheres to Gp1b on platelets Homozygotes resemble hemophilia A
42
VWF Laboratory Testing
APTT - prolonged BT - prolonged PT - normal TT - normal Fibrinogen - normal Platelet Count - normal to decreased 8:c, VWF:Ag, VWF:Rco - decreased Platelet aggregation to ristocetin - abnormal
43
VWF Therapy
Cryoprecipitate DDAVP (type 1)
44
Factor 9 Inheritance Hemophilia B
Sex-linked Recessive
45
Factor 9 Clinical Presentation
Bleeding (may be similar to Hemophilia A) May develop inhibitor
46
Factor 9 Laboratory Testing
APTT - prolonged PT - normal Fibrinogen concentration - normal BT - normal TT - normal
47
Factor 9 Therapy
Factor 9 concentrates
48
Factor 10 Inheritance
AR Inheritance Acquired with amyloidosis
49
Factor 10 Clinical Presentation
Bleeding
50
Factor 10 Laboratory Testing
PT - prolonged APTT - prolonged ST - prolonged Fibrinogen concentration - normal BT - normal TT - normal
51
Factor 10 Therapy
Prothrombin Complex concentrates FFP
52
Factor 11 Inheritance
AR Inheritance Mainly in Jewish population
53
Factor 11 Clinical Presentation
Bleeding
54
Factor 11 Laboratory Testing
APTT - prolonged PT - normal Fibrinogen concentration - normal BT - normal TT - normal
55
Factor 11 Therapy
FFP
56
Factor 12 Inheritance
AR Inheritance
57
Factor 12 Clinical Presentation
Thrombosis
58
Factor 12 Laboratory Testing
APTT - prolonged PT - normal Fibrinogen concentration - normal BT - normal TT - normal
59
Factor 12 Therapy
Treat thrombosis FFP
60
Prekallekrein (PK) Inheritance
AR Inheritance
61
PK Clinical Presentation
Thrombosis
62
PK Laboratory Testing
APTT - prolonged PT - normal Fibrinogen concentration - normal BT - normal TT - normal
63
PK Therapy
Treat thrombosis FFP
64
High Molecular Weight Kininogen (HMWK) Inheritance
AR Inheritance
65
HMWK Clinical Presentation
Thrombosis
66
HMWK Laboratory Testing
APTT - prolonged PT - normal Finbrinogen concentration - normal BT - normal TT - normal
67
HMWK Therapy
Treat thrombosis FFP
68
Factor 13 Inheritance
AR Inheritance
69
Factor 13 Clinical Presentation
Bleeding (delayed, umbilical cord) Poor wound healing
70
Factor 13 Testing
PT - normal APTT - normal Fibrinogen concentration - normal BT - normal TT - normal Clot solubility test - abnormal
71
Factor 13 Therapy
FFP
72
Alpha 2 antiplasmin Inheritance
AR Inheritance
73
Alpha 2 antiplasmin Clinical Presentation
Bleeding - not delayed
74
Alpha 2 antiplasmin Laboratory Testing
PT - normal APTT - normal Fibrinogen concentration - normal BT - normal TT - normal
75
Alpha 2 antiplasmin Therapy
FFP

Coagulation (6 decks)

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