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HEMA 256 > Module 4: Disorders of Hemostasis > Flashcards

Module 4: Disorders of Hemostasis Flashcards

1
Q

Vascular and extravascular disorders are due to:

A

a defect in the structure or function of the vascular endothelium or sub endothelium (collagen)

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2
Q

In vascular and extravascular disorders, platelet count, PT and PTT would be:

A

normal

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3
Q

In vascular and extravascular disorders, bleeding time or closure time would be:

A

prolonged

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4
Q

Acquired vascular disorders include:

A

Senile purapura
Simple easy bruising
Secondary vascular purapuras

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5
Q

What is senile purapura?

A

Bruising in the aged, atrophy and degeneration of the connective tissue

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6
Q

Inherited vascular disorders include:

A 
Hereditary hemorrhagic telangiectasia
Ehlers-Danlos syndrome
Marfans Syndrome
Osteogeneis imperfecta
Homocystienuria
Pseudo Xanthoma Elasticum
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7
Q

Thrombocytopenia means:

A

Decreased platelet count

Critical, less than 20 x 10^9/L

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8
Q

Thrombocytosis means:

A

Increased platelet count

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9
Q

Thrombocythemia means:

A

Extreme increased platelet count >1000 x 10^9/L

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10
Q

Lab findings in thrombocytopenia:

A

Decreased platelet count
Prolonged bleeding time
Prolonged closure time
Poor clot retraction

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11
Q

Thrombocytopenia is caused by:

A

Decreased or ineffective production of platelets
Increased destruction or utilization of platelets
Abnormal distribution

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12
Q

What happens in marrow hypoplasia?

A

All cells are decreased, decreased megakaryocytes in the bone marrow

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13
Q

What happens in ineffective megakaryopoiesis?

A

Megakaryocytes fail to survive or to normally release platelets (low platelet count)
Bone marrow appears normal

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14
Q

What is hereditary hemorrhagic telangiectasia?

A

Autosomal dominant defect in the subendothelial collagen

Results in dilation of capillaries, loss of vascular patency (petechiae), spontaneous bleeds from mucous membranes

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15
Q

What is Ehlers-Danlos Syndrome?

A

Inherited disorder

Loss of elasticity in the epidermis and sub epidermal tissues

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16
Q

What is Marfan Syndrome?

A

Defect in chromosome 15

Abnormal fibrillan in connective tissue and weakness

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17
Q

What is Osteogenesis Imperfecta?

A

Genetic disorder of defective collagen formation characterized by bones that break easily

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18
Q

What is homocystinuria?

A

Inherited disorder of the metabolism of the amino acid methionine

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19
Q

What Pseudo Xanthoma Elasticum?

A

Inherited disorder of elastin in which elastic tissues in the body (skin, vessels, retina) become mineralized, especially with calcium

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20
Q

Secondary vascular purapuras are:

A

endothelial damage from a variety of causes (immune damage, vitamin C deficiency, endotoxins, liver disease, etc)

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21
Q

Clinical findings in thrombocytopenia:

A

Skin purapura
Mucosal hemorrhages
Increased bleeding after trauma

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22
Q

Conditions causing excessive consumption of platelets

A

Thrombotic thrombocytopenic purapura (TTP)
Disseminated intravascular coagulation (DIC)
Hemolytic uremic syndrome (HUS)

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23
Q

What is thrombotic thrombocytopenic purapura (TTP)?

A

Disorder caused by a deficiency of the metalloprotease enzyme ADAMTS-13 (required to break up ultra-large vWF multimers to smaller, less adhesive multimers)

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24
Q

Lab findings in TTP

A

Increased megakaryocytes in the bone marrow
Increased megathrombocytes in the blood smear
Increased platelet aggregation in blood film
Decreased platelet survival (radioisotope method)

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25
Q

What is immune thrombocytopenic purapura (ITP)?

A

Platelet antibody (usually GPIIb/IIIa or GPIb) sensitizes the platelets causing premature removal by macrophages
Usually secondary to viral infections or drugs
May be acute (rare, in children, usually after vaccination or viral infection) or chronic

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26
Q

Lab findings in ITP

A

Increased megakaryocytes in the bone marrow
Platelet antibody in serum
Decreased platelet survival

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27
Q

Lab findings in thrombocytosis

A

Increased platelet count with abnormal morphology
Increased megathrombocytes
Increased red cell fallout in the clot retraction test
Normal or prolonged bleeding time

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28
Q

Abnormal platelet function is indicated by:

A

skin and mucosal hemorrhage with prolonged bleeding time and normal platelet count

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29
Q

Storage pool diseases (granule defects)

A

Secondary aggregation disorder

Deficiency of dense and/or alpha granules

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30
Q

Primary secretion defects (enzymatic pathway defects)

A

Deficiencies of enzymes and other secondary messengers that transmit signals from surface receptors to cause the release of the granule contents
Resemble storage pool deficiencies but no quantity or content abnormalities

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31
Q

Lab findings in primary secretion defects

A

Bleeding time usually prolonged
Platelet count normal
Other results variable depending on cause

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32
Q

What is thrombasthenia (Glanzmann disease)?

A

Inherited (autosomal recessive)
Failure of primary aggregation caused by reduced amounts of GP IIb/IIIa
Fibrinogen cannot be bound to the platelet membrane

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33
Q

Lab findings in thrombasthenia (Glanzmann disease)

A

Bleeding time greatly prolonged
Clot retraction defective
Platelet adhesion to collagen is normal
Platelet aggregation abnormal with ADP, epinephrine, thrombin, and serotonin, normal with ristocetin

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34
Q

What is Bernard-Soulier Syndrome (BSS)?

A

Also called Giant Platelet Syndrome
Autosomal recessive
Failure of platelet adhesion caused by reduced amounts of membrane GP Ib/IX
vWF cannot be bound to the platelet membrane

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35
Q

Lab findings in thrombasthenia (Glanzmann disease)

A 
Platelet count - normal
Platelet morphology - normal
Closure time/bleeding time - prolonged
Aggregation with ristocetin - normal
Glycoprotein defect - GP IIb/IIIa
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36
Q

Lab findings in Bernard-Soulier Syndrome

A 
Platelet count - decreased/normal
Platelet morphology - giant
Closure time/bleeding time - prolonged
Aggregation with ristocetin - abnormal
Glycoprotein defect - GP Ib/IX
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37
Q

Most common acquired platelet defect is a result of:

A

aspirin

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38
Q

The enzyme that is inhibited by aspirin:

A

cyclooxygenase

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39
Q

Coagulation disorders are due to:

A

Failure of synthesis
Production of abnormal molecules
Excessive destruction of consumption of factors
Inactivation of factors by circulating anticoagulants

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40
Q

PT tests factors:

A

Extrinsic

I, II, V, VII, X

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41
Q

APTT tests factors:

A

Intrinsic

All factors except III, VII, XIII

42
Q

Severity of the deficiency is proportional to the bleed for factors:

A

I, II, V, VIII, IX, X

43
Q

Patients with a severe factor deficiency would have:

A

Less than 1% of the normal level

Severe bleeding symptoms, spontaneous hemorrhages

44
Q

Patients with a moderate factor deficiency would have:

A

1-5% of normal level

Occasional spontaneous hemorrhages, severe bleeding after minor injury

45
Q

Patients with a mild factor deficiency would have:

A

5-25% of normal level

Severe bleeding after surgery, some bleeding after mild trauma

46
Q

Deficiency of what factors causes prolonged APTT but no clinical bleeding?

A

XII, HMWK, PK

47
Q

What factors cause a disproportionate mild bleed?

A

XI, VII

48
Q

What factor causes a disproportionate severe bleed?

A

XIII

49
Q

Which is the most common bleeding disorder?

A

von Willebrands Disease

50
Q

Which is the most common hemophilia?

A

A

51
Q

Factor deficient in hemophilia A:

A

VIIIc

52
Q

Factor deficient in hemophilia B:

A

IX

53
Q

Gene affected in hemophilia A:

A

Xq28

54
Q

Gene affected in hemophilia B:

A

Xq27

55
Q

Therapy for hemophilia A:

A

Factor VIII infusion

56
Q

Therapy for hemophilia B:

A

Prothrombin group concentrate or IX concentrate

57
Q

Complication in hemophilia A:

A

Development of immune VIII antibodies

58
Q

Which hemophilia therapy lasts longer and why?

A

IX because of its longer half-life

59
Q

What type of disorder is von Willebrands Disease?

A

Platelet adhesion

60
Q

What chromosome is affected in von Willebrands Disease?

A

12

61
Q

What is the treatment for von Willebrands Disease?

A

VIII

62
Q

Most useful tests for workup of vWF?

A

Platelet agglutination with ristocetin

Platelet aggregation with ristocetin (reduced)

63
Q

Type 1 von Willebrands Disease

A

Partial quantitative deficiency of vWF

Most common type of vWD

64
Q

Type 2 von Willebrands Disease

A

Qualitative deficiency of vWF

65
Q

Type 3 von Willebrands Disease

A

Severe quantitative deficiency of vWF

66
Q

Lab findings in hemophilia A

A

Platelet count - normal
Bleeding time - normal
PT - normal
APTT - prolonged

67
Q

Lab findings in hemophilia B

A

Platelet count - normal
Bleeding time - normal
PT - normal
APTT - prolonged

68
Q

Lab findings in vWD type 1

A

Platelet count - normal
Bleeding time - usually prolonged
PT - normal
APTT - usually normal by may be prolonged

69
Q

Circulating abnormal anticoagulants and antifactors are:

A

Specific factor antibodies

Non-specific inhibitors: “antiphospholipid antibody” (lupus anticoagulant, anticardiolipin antibody)

70
Q

Test used to differentiate deficiency from circulating anticoagulants or antifactors:

A

Mixing study

71
Q

What happens in vitamin K deficiency?

A

Factors are not carboxylated and cannot bind calcium

72
Q

Factors affected by vitamin K deficiency

A

Prothrombin group - II, VI, IX, X

Protein C and S

73
Q

PIVKA stands for:

A

Proteins induced by vitamin K absence = non-carboxylated. inactive abnormal prothrombin factor molecules

74
Q

What oral anticoagulant is an antagonist?

A

Warfarin (coumadin)

75
Q

Liver disease results in reduced production of what factors?

A

I, II, V, VII, IX, X

76
Q

2 liver disorders affecting coagulation:

A 
Obstructive jaundice (cannot absorb vitamin k)
Severe hepatitis (damaged cells produces less factors)
77
Q

Destruction of liver cells may results in:

A

DIC (coagulation factors are not removed)
Release of increased amounts of tPA from hepatic cells triggering fibrinolysis
Splenomegaly increasing splenic sequestering of platelets

78
Q

DIC (Disseminated Intravascular Coagulation) is:

A

Condition of widespread intravascular coagulation and secondary fibrinolysis

79
Q

DIC results in:

A

Consumption and destruction of coagulation factors until they are deficient
Thrombocytopenia
Abnormal bleeding

80
Q

DIC lab findings should confirm:

A

Activation of coagulation
Activation of fibrinolysis
Inhibitor consumption
End organ damage or failure

81
Q

Main lab results in DIC:

A

Factor deficiencies (therefore prolonged PT and PTT)
Thrombocytopenia
Increased fibrin and fibrinogen degradation products
Increased D-dimer
Schistocytes

82
Q

Disorders of fibrinolysis

A

Primary
Secondary
Therapeutic
Impaired

83
Q

What is primary fibrinolysis?

A

Activation of fibrinolysis in the absence of coagulation
Abnormal activation of plasminogen to plasmin which destroys fibrinogen
No FDP because no coagulation has taken place for fibrin to be formed from the fibrinogen

84
Q

What is secondary fibronolysis?

A

Occurs when fibrin is deposited (normal)

Abnormal increase in DIC

85
Q

What is therapeutic fibrinolysis?

A

Induced by injection of direct plasminogen activators

86
Q

What is impaired fibrinolysis?

A

Due to decreased production, increased destruction, or increased inhibition of plasminogen, plasminogen activators, or plasmin

87
Q

Fibrinogen degradation product test and D-dimer test (fibrin degradation products) in primary fibrinolysis:

A

FDP: pos

D-dimer: neg

88
Q

Fibrinogen degradation product test and D-dimer test (fibrin degradation products) in secondary fibrinolysis:

A

FDP: pos

D-dimer: pos

89
Q

Thrombosis is:

A

abnormal formation in circulation of solid, localized masses of fibrin and/or platelets that cause partial or complete vessel blockage

90
Q

Thromboembolism is:

A

thrombi break away and block smaller vessels

91
Q

3 factors involved in forming a thrombus are:

A

Slowed blood flow
Hypercoagulability
Vessel wall damage

92
Q

Inherited disorders that promote thrombosis include:

A 
Factor V Leiden gene mutation (protein C resistance)
Antithrombin III deficiency
Protein C or S deficiency
Homocysteinuria
Dysfibrinogenemia
Abnormal plasminogen
Prothrombin G20210A variant
93
Q

What is Factor V Leiden gene mutation (protein C resistance)?

A

Most common cause of increased risk of venous thrombosis
Produce abnormal factor V that resists that action for activated protein C
Factor is inappropriately maintained in an active form and continues to promote coagulation

94
Q

What is antithrombin III deficiency?

A

Normally works with heparin to inhibit factors

Inadequate inhibition of thrombin, Xa, IXa

95
Q

What is Protein C or S deficiency?

A

Inadequate inhibition of V and VIII

Impaired activation of fibrinolysis because activated protein C inhibits antiplasmin

96
Q

What is homocysteinuria?

A

Damaged endothelium by deposits of amino acids causes activation of coagulation and atheroschlerosis

97
Q

What is dysfibrinogenemia?

A

Abnormal fibrinogen makes abnormal fibrin that will not neutralize thrombin

98
Q

What is abnormal plasminogen?

A

Impaired formation of plasmin

99
Q

Prothrombin G20210A variant

A

Abnormal allele of factor II = increased FII levels = increased thrombosis

100
Q

What immune condition is an acquired coagulation deficiency and also and acquired disorder that promotes thrombosis?

A

Lupus anticoagulant

101
Q

Lupus anticoagulant

A

Antibody that binds to phospholipids and proteins associated with the cell membrane
Interferes with interactions between cell membrane and clotting factors necessary for proper cascade function
Anticoagulant in vitro, coagulant in vivo (in the body)

HEMA 256 (5 decks)

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