Hemostasis Flashcards

1
Q

steps in hemostasis

A

1) blood vessels constrict to slow blood flow
2) platelets aggregate and stick to injured area and to each other
3) coag factors reinforce platelet plug with fibrin clot
4) removal of excess hemostatic material re-establishes integrity

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2
Q

function of vascular system in hemostasis

A

vasoconstriction, diversion of blood flow, initiate contact with platelets and coag proteins

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3
Q

function of platelets in hemostasis

A

formation of platelet plug to stop initial bleeding

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4
Q

what stabilizes platelet plug

A

fibrin formation and thromboplastin

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5
Q

fibrinogen group of coagulation factors

A

I
V
VIII
XIII

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6
Q

prothrombin group of coagulation factors

A

II
VII
IX
X

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7
Q

vitamin K dependent coagulation factors

A

II
VII
IX
X

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8
Q

fibrinogen is factor ___

A

I

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9
Q

prothrombin is factor ___

A

II

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10
Q

tissue thromboplastin/tissue factor is factor ___

A

III

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11
Q

ionic calcium is factor ___

A

IV

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12
Q

labile factor/proaccelerin is factor ___

A

V

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13
Q

stable factor (procovertin) is factor ___

A

VII

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14
Q

antihemophilic factor/von Willebrand factor is factor ___

A

VIII

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15
Q

Christmas factor/plasma thromboplastin factor ___

A

IX

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16
Q

Stuart-Prower factor ___

A

X

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17
Q

plasma thromboplastin antecedent is factor ___

A

XI

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18
Q

Hageman factor/contact factor ___

A

XII

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19
Q

fibrin stabilizing factor ___

A

XIII

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20
Q

product recommended for increased LY30

A

antifibrinolytic (TXA)

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21
Q

product recommended for decreased MA

A

platelet

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22
Q

product recommended for decreased angle and increased K

A

cryo

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23
Q

product recommended for increased R

A

FFP

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24
Q

symptoms of vitamin K deficiency

A
easy bruising
oozing from nose or gums
excessive bleeding
heavy periods
bleeding from GI tract
blood in urine/stool
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25
Q

coag test that measures time it takes for plasma to clot once thromboplastin and calcium are added

A

prothrombin time

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26
Q

PT measures what pathway?

A

extrinsic and common

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27
Q

coag test used to monitor warfarin therapy

A

PT

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28
Q

conditions with prolonged PT

A
warfarin therapy
vitamin K deficiency
liver disease
acquired or hereditary factor deficiency
factor inhibitor drugs or antibodies
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29
Q

coag test that measures times it takes for plasma to clot once activator reagent is added

A

activated partial thromboplastin time

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30
Q

PTT measures deficiencies in what pathway

A

intrinsic and common

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31
Q

PT reference range

A

11-13.5 seconds

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32
Q

PTT reference range

A

30-40 seconds

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33
Q

coag test used to monitor heparin therapy

A

PTT

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34
Q

conditions with prolonged PTT

A

heparin therapy
circulating inhibitors
decreases in circulating levels of II, IX, X in patients on oral anticoagulants

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35
Q

precursor to fibrin which is necessary for clot formation

A

fibrinogen/factor I

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36
Q

enzyme that converts fibrinogen to fibrin

A

thrombin

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37
Q

normal fibrinogen level

A

200-400 mg/dl

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38
Q

how is fibrinogen level measured

A

excess thrombin added to plasma and clotting time measured

clotting time inversely proportional to fibrinogen concentration

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39
Q

test that measures conversion of fibrinogen to fibrin

A

thrombin time

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40
Q

normal thrombin time

A

15-25 seconds

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41
Q

reasons for prolonged thrombin time

A

low fibrinogen
impaired fibrinogen function
thrombin inhibitors
multiple myeloma

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42
Q

test to aid in determining cause of abnormal/prolonged PT or PTT results in which normal fresh plasma is mixed 1:1 with patient plasma

A

mixing study

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43
Q

if PT/PTT is corrected in a mixing study, the prolong is due to ___

A

factor deficiency

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44
Q

is PT/PTT is not corrected in a mixing study the prolong is due to ___

A

potential factor inhibitor

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45
Q

detects how well platelets stick together

A

Platelet (Ristocetin) aggregation

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46
Q

abnormal ristocetin aggregation due to:

A
meds that affect platelet function
von Willebrand disease
liver disease
acute leukemia
food consumption (caffeine, garlic, ginger)
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47
Q

test to aid in determining cause of abnormal PT/PTT where patient’s plasma is tested with specific factor deficient substrate plasma

A

factor assay

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48
Q

factors tested using PT methodology when doing factor assays

A

II
V
X

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49
Q

factors tested using PTT methodology when doing factor assays

A

VIII
IX
XI
XII

50
Q

test that screens for factor XIII deficiency

A

5M urea lysis (urea solubility)

51
Q

physiology of 5M urea test

A
factor XIII crosslinks fibrin to stabilize clot
weak clot (fibrin not crosslinked) = clot dissolves
normal clots = do not dissolve
52
Q

steps in fibrinolysis

A

plasminogen –> plasmin –> breaks down either fibrin or fibrinogen –> fibrin degradation products

53
Q

function of antithrombin III

A

blocks abnormal clot formation

54
Q

fibrin degradation product that allows differentiation between normal fibrinolysis and abnormal clot formation

A

D-Dimer

55
Q

normal D-dimer

A

<0.5 mg/ml

56
Q

elevated D-dimer indicates excessive ____

A

clot formation

57
Q

classic reason for elevated D-dimer

A

DIC

58
Q

continual activation of clotting and fibrinolytic cascades

A

disseminated intravascular coagulation

59
Q

Lab values in DIC

A
Prolonged PT and PTT
Decreased fibrinogen
Increased D-dimer
Increased thrombin time
Low platelet count
60
Q

Hemophilia A is deficiency of?

A

Factor VIII:C

61
Q

Which hemophilia is “classic” hemophilia?

A

Hemophilia A

62
Q

Inheritance pattern of hemophilia A

A

Sex-linked recessive (X chromosome)

63
Q

Severe classification of hemophilia A

A

Less than 1% factor VIII activity
Severe bleeding
Crippling
Needs treatment

64
Q

Moderate classification of hemophilia A

A

1-5% factor VIII activity
Occasional hemarthrosis
Treatment needed post-trauma

65
Q

Mild classification of hemophilia A

A

6-40% factor VIII activity
Rare hemarthrosis
Fairly normal life
Severe bleeding after dental extraction or other minor surgery

66
Q

Hemophilia A lab results

A
Prolonged PTT
FVIII decreased
PT normal
Platelet count normal
Ristocetin aggregation normal
67
Q

Treatment for hemophilia A

A

Recombinant FVIII
Human plasma derived FVIII
DDAVP for mild cases

68
Q

Use of Cryo for factor VIII deficiency

A

No longer used due to excessive donor exposure

69
Q

Deficiency in von Willebrand disease

A

VIII:vWF

70
Q

Difference in deficiency in hemophilia A and von Willebrand disease

A

Abnormality of FVIII complex different

71
Q

Most common factor deficiency

A

Von Willebrand

72
Q

Von Willebrand bleeding risk when factor activity level is < ___

A

40%

73
Q

Blood type correlation with von Willebrand deficiency

A

Levels vary with blood type

AB > B > A > O

74
Q

Von Willebrand factor is carrier molecule for what factor

A

VIII

75
Q

Symptoms of von Willebrand disease

A

Mucosal and cutaneous bleeds
Easy bruising
GI bleeding
Menorrhagia

76
Q

Why is von Willebrand factor essential for platelet function?

A

Interaction with endothelium

77
Q

Lab results in von Willebrand disease

A
PTT prolonged
FVIII decreased
PT normal
Ristocetin aggregation decreased
platelet count normal
78
Q

Treatment of von Willebrand disease

A

DDAVP
Cryo
Human plasma derived VIII (must contain both FVIII and vWF)

79
Q

Why is recombinant/monoclonal FVIII not used for von Willebrand disease

A

Does not contain vWF

80
Q

Christmas disease is which hemophilia?

A

B

81
Q

Factor deficiency in hemophilia B

A

IX

82
Q

Inheritance pattern for hemophilia B

A

sex-linked recessive

83
Q

Which hemophilia is more common

A

A

84
Q

Hemophilia B lab results

A

PTT prolonged
FIX decreased
PT normal
Platelet count normal

85
Q

Treatment for hemophilia B

A

Plasma derived factor IX

Recombinant factor IX

86
Q

Factors contained in plasma derived prothrombin complex FIX

A

II
VII
IX
X

87
Q

Factor I half life

A

72-120 hours

88
Q

Factor V half life

A

12-36 hours

89
Q

Factor VII half life

A

5-8 hours

90
Q

Factor VIII half life

A

8-12 hours

91
Q

Von Willebrand half life

A

22-40 hours

92
Q

Factor IX half life

A

48-72 hours

93
Q

Factor X half life

A

48-60 hours

94
Q

Factor XIII half life

A

72-120 hours

95
Q

How is factor dosage calculated?

A

1 unit of factor per kg body weight should raise plasma factor level by ~2%

96
Q

Half life application if half life is 8-12 hours

A

~8 hours after infusion the plasma level will drop by 50%

If starting level is 150%, in 8 hours level will be 75%

In 16 hours level will be 37.5%

In 24 hours level will be 18.75%

97
Q

Causes of fibrinogen deficiency

A
Liver disease
Multiple myeloma
Malignancy
Inhibitors
DIC
Surgery
OB cases
Drugs
98
Q

Lab results of fibrinogen deficiency

A

Prolonged PT
Prolonged PTT
Decreased fibrinogen

99
Q

Treatment of fibrinogen deficiency

A

Treat underlying cause

Cryo supportive therapy

100
Q

Prothrombin deficiency causes

A
Vitamin K deficiency
Liver disease
DIC
GI disease
Obstructive jaundice
Coumarin drug intoxication
101
Q

Prothrombin deficiency lab results

A

Prolonged PT
Prolonged PTT
Decreased FII assay

102
Q

Treatment for prothrombin deficiency

A

Vitamin K
Treat underlying cause
FFP supportive therapy

103
Q

Factor V deficiency causes

A
Inherited Owren’s disease
Parahemophilia
Inhibitors
DIC
Autoimmune disease
104
Q

Factor V deficiency lab results

A

Prolonged PT
Prolonged PTT
Decreased FV assay

105
Q

Treatment for factor V deficiency

A

Treat underlying cause

FFP supportive therapy

106
Q

Factor VII deficiency causes

A

Inhibitors
Liver disease
Coumarin drug therapy
Vitamin K deficiency

107
Q

Factor VII deficiency lab results

A

Prolonged PT
Prolonged PTT
Decreased FVII assay

108
Q

Treatment for factor VII deficiency

A

Commercial FVII concentrate
FFP supportive therapy
Vitamin K (unless hereditary VII deficiency)

109
Q

Factor X deficiency causes

A

Stuart or Stuart-Prower disease

110
Q

Factor X deficiency lab results

A

Prolonged PT
Prolonged PTT
Decreased FX assay

111
Q

Treatment of factor X deficiency

A

FFP supportive therapy

Vitamin K unless hereditary deficiency

112
Q

Factor XI deficiency causes

A

Autosomal recessive disorder, more common in Jewish families

113
Q

Factor XI deficiency lab results

A

Normal PT
Prolonged PTT
Decreased FXI assay

114
Q

Treatment for factor XI deficiency

A

Usually not necessary

May give FFP if bleeding in surgical situations

115
Q

Factor XII deficiency causes

A

Rare autosomal recessive disorder

May exhibit thrombotic episodes later in life

116
Q

Factor XII deficiency lab results

A

Normal PT
Prolonged PTT
FXII activated by glass in vivo

117
Q

Treatment of factor XII deficiency

A

Not necessary even in surgical bleeding

118
Q

Factor XIII deficiency causes

A
Umbilical cord bleeding
Poor wound healing
Spontaneous abortions
Abnormal scar formation
Intracranial hemorrhage
Oozing from wounds after 36 hours
119
Q

Factor XIII deficiency lab results

A

Normal PT
Normal PTT
Normal platelet count
Soluble in 5M urea

120
Q

Treatment for factor XIII deficiency

A

Cryo supportive therapy