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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
Q

Factor 5 Leiden Laboratory Testing

A

Modified APTT
PCR

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26
Q

Factor 5 Leiden Therapy

A

Anticoagulant Therapy

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27
Q

Factor 7 Inheritance

A

AR Inheritance

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28
Q

Factor 7 Clinical Presentation

A

Bleeding

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29
Q

Factor 7 Laboratory Testing

A

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

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30
Q

Factor 7 Therapy

A

FFP
Prothrombin complex concentrate

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31
Q

Factor 10 Inheritance

A

AR Inheritance
May be acquired with amyloidosis (vessels and organs)

32
Q

Factor 10 Laboratory Testing

A

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

33
Q

Factor 10 Therapy

A

FFP
Prothrombin complex concentrate

34
Q

Factor 8 Inheritance
Hemophilia A

A

Sex-linked Recessive
- women are carriers

35
Q

Factor 8:c Clinical Presentation

A

Bleeding
- Severe (0-1%), Moderate (2-5%), Mild (5-20%)

36
Q

Factor 8:c Laboratory Testing

A

APTT - prolonged
PT - normal
BT - normal
TT - normal
Fibrinogen - normal
8:c - decreased
VWF:Ag - normal or increased
VWF:Rco - normal or increased

37
Q

Factor 8:c Therapy

A

Factor 8 concentrates
Counseling

38
Q

Factor 8:c Considerations

A

Inhibitors
- Time and Temperature dependent
- Bethesda Titer
- No response to therapy
- Alternative therapies

39
Q

VWF Inheritance

A

AD Inheritance

40
Q

VWF Types of disease

A

Type 1 - Normal structure, defective release
Type 2 - Abnormal structure
Type 3 - Absent synthesis

41
Q

VWF Clinical Presentation

A

Bleeding (mucosal)
VWF adheres to Gp1b on platelets
Homozygotes resemble hemophilia A

42
Q

VWF Laboratory Testing

A

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
Q

VWF Therapy

A

Cryoprecipitate
DDAVP (type 1)

44
Q

Factor 9 Inheritance
Hemophilia B

A

Sex-linked Recessive

45
Q

Factor 9 Clinical Presentation

A

Bleeding (may be similar to Hemophilia A)
May develop inhibitor

46
Q

Factor 9 Laboratory Testing

A

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

47
Q

Factor 9 Therapy

A

Factor 9 concentrates

48
Q

Factor 10 Inheritance

A

AR Inheritance
Acquired with amyloidosis

49
Q

Factor 10 Clinical Presentation

50
Q

Factor 10 Laboratory Testing

A

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

51
Q

Factor 10 Therapy

A

Prothrombin Complex concentrates
FFP

52
Q

Factor 11 Inheritance

A

AR Inheritance
Mainly in Jewish population

53
Q

Factor 11 Clinical Presentation

54
Q

Factor 11 Laboratory Testing

A

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

55
Q

Factor 11 Therapy

56
Q

Factor 12 Inheritance

A

AR Inheritance

57
Q

Factor 12 Clinical Presentation

A

Thrombosis

58
Q

Factor 12 Laboratory Testing

A

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

59
Q

Factor 12 Therapy

A

Treat thrombosis
FFP

60
Q

Prekallekrein (PK) Inheritance

A

AR Inheritance

61
Q

PK Clinical Presentation

A

Thrombosis

62
Q

PK Laboratory Testing

A

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

63
Q

PK Therapy

A

Treat thrombosis
FFP

64
Q

High Molecular Weight Kininogen (HMWK) Inheritance

A

AR Inheritance

65
Q

HMWK Clinical Presentation

A

Thrombosis

66
Q

HMWK Laboratory Testing

A

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

67
Q

HMWK Therapy

A

Treat thrombosis
FFP

68
Q

Factor 13 Inheritance

A

AR Inheritance

69
Q

Factor 13 Clinical Presentation

A

Bleeding (delayed, umbilical cord)
Poor wound healing

70
Q

Factor 13 Testing

A

PT - normal
APTT - normal
Fibrinogen concentration - normal
BT - normal
TT - normal
Clot solubility test - abnormal

71
Q

Factor 13 Therapy

72
Q

Alpha 2 antiplasmin Inheritance

A

AR Inheritance

73
Q

Alpha 2 antiplasmin Clinical Presentation

A

Bleeding - not delayed

74
Q

Alpha 2 antiplasmin Laboratory Testing

A

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

75
Q

Alpha 2 antiplasmin Therapy

Coagulation (6 decks)

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