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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
What is immune thrombocytopenic purapura (ITP)?
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
26
Lab findings in ITP
Increased megakaryocytes in the bone marrow Platelet antibody in serum Decreased platelet survival
27
Lab findings in thrombocytosis
Increased platelet count with abnormal morphology Increased megathrombocytes Increased red cell fallout in the clot retraction test Normal or prolonged bleeding time
28
Abnormal platelet function is indicated by:
skin and mucosal hemorrhage with prolonged bleeding time and normal platelet count
29
Storage pool diseases (granule defects)
Secondary aggregation disorder | Deficiency of dense and/or alpha granules
30
Primary secretion defects (enzymatic pathway defects)
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
31
Lab findings in primary secretion defects
Bleeding time usually prolonged Platelet count normal Other results variable depending on cause
32
What is thrombasthenia (Glanzmann disease)?
Inherited (autosomal recessive) Failure of primary aggregation caused by reduced amounts of GP IIb/IIIa Fibrinogen cannot be bound to the platelet membrane
33
Lab findings in thrombasthenia (Glanzmann disease)
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
34
What is Bernard-Soulier Syndrome (BSS)?
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
35
Lab findings in thrombasthenia (Glanzmann disease)
``` Platelet count - normal Platelet morphology - normal Closure time/bleeding time - prolonged Aggregation with ristocetin - normal Glycoprotein defect - GP IIb/IIIa ```
36
Lab findings in Bernard-Soulier Syndrome
``` Platelet count - decreased/normal Platelet morphology - giant Closure time/bleeding time - prolonged Aggregation with ristocetin - abnormal Glycoprotein defect - GP Ib/IX ```
37
Most common acquired platelet defect is a result of:
aspirin
38
The enzyme that is inhibited by aspirin:
cyclooxygenase
39
Coagulation disorders are due to:
Failure of synthesis Production of abnormal molecules Excessive destruction of consumption of factors Inactivation of factors by circulating anticoagulants
40
PT tests factors:
Extrinsic | I, II, V, VII, X
41
APTT tests factors:
Intrinsic | All factors except III, VII, XIII
42
Severity of the deficiency is proportional to the bleed for factors:
I, II, V, VIII, IX, X
43
Patients with a severe factor deficiency would have:
Less than 1% of the normal level | Severe bleeding symptoms, spontaneous hemorrhages
44
Patients with a moderate factor deficiency would have:
1-5% of normal level | Occasional spontaneous hemorrhages, severe bleeding after minor injury
45
Patients with a mild factor deficiency would have:
5-25% of normal level | Severe bleeding after surgery, some bleeding after mild trauma
46
Deficiency of what factors causes prolonged APTT but no clinical bleeding?
XII, HMWK, PK
47
What factors cause a disproportionate mild bleed?
XI, VII
48
What factor causes a disproportionate severe bleed?
XIII
49
Which is the most common bleeding disorder?
von Willebrands Disease
50
Which is the most common hemophilia?
A
51
Factor deficient in hemophilia A:
VIIIc
52
Factor deficient in hemophilia B:
IX
53
Gene affected in hemophilia A:
Xq28
54
Gene affected in hemophilia B:
Xq27
55
Therapy for hemophilia A:
Factor VIII infusion
56
Therapy for hemophilia B:
Prothrombin group concentrate or IX concentrate
57
Complication in hemophilia A:
Development of immune VIII antibodies
58
Which hemophilia therapy lasts longer and why?
IX because of its longer half-life
59
What type of disorder is von Willebrands Disease?
Platelet adhesion
60
What chromosome is affected in von Willebrands Disease?
12
61
What is the treatment for von Willebrands Disease?
VIII
62
Most useful tests for workup of vWF?
Platelet agglutination with ristocetin | Platelet aggregation with ristocetin (reduced)
63
Type 1 von Willebrands Disease
Partial quantitative deficiency of vWF | Most common type of vWD
64
Type 2 von Willebrands Disease
Qualitative deficiency of vWF
65
Type 3 von Willebrands Disease
Severe quantitative deficiency of vWF
66
Lab findings in hemophilia A
Platelet count - normal Bleeding time - normal PT - normal APTT - prolonged
67
Lab findings in hemophilia B
Platelet count - normal Bleeding time - normal PT - normal APTT - prolonged
68
Lab findings in vWD type 1
Platelet count - normal Bleeding time - usually prolonged PT - normal APTT - usually normal by may be prolonged
69
Circulating abnormal anticoagulants and antifactors are:
Specific factor antibodies | Non-specific inhibitors: "antiphospholipid antibody" (lupus anticoagulant, anticardiolipin antibody)
70
Test used to differentiate deficiency from circulating anticoagulants or antifactors:
Mixing study
71
What happens in vitamin K deficiency?
Factors are not carboxylated and cannot bind calcium
72
Factors affected by vitamin K deficiency
Prothrombin group - II, VI, IX, X | Protein C and S
73
PIVKA stands for:
Proteins induced by vitamin K absence = non-carboxylated. inactive abnormal prothrombin factor molecules
74
What oral anticoagulant is an antagonist?
Warfarin (coumadin)
75
Liver disease results in reduced production of what factors?
I, II, V, VII, IX, X
76
2 liver disorders affecting coagulation:
``` Obstructive jaundice (cannot absorb vitamin k) Severe hepatitis (damaged cells produces less factors) ```
77
Destruction of liver cells may results in:
DIC (coagulation factors are not removed) Release of increased amounts of tPA from hepatic cells triggering fibrinolysis Splenomegaly increasing splenic sequestering of platelets
78
DIC (Disseminated Intravascular Coagulation) is:
Condition of widespread intravascular coagulation and secondary fibrinolysis
79
DIC results in:
Consumption and destruction of coagulation factors until they are deficient Thrombocytopenia Abnormal bleeding
80
DIC lab findings should confirm:
Activation of coagulation Activation of fibrinolysis Inhibitor consumption End organ damage or failure
81
Main lab results in DIC:
Factor deficiencies (therefore prolonged PT and PTT) Thrombocytopenia Increased fibrin and fibrinogen degradation products Increased D-dimer Schistocytes
82
Disorders of fibrinolysis
Primary Secondary Therapeutic Impaired
83
What is primary fibrinolysis?
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
What is secondary fibronolysis?
Occurs when fibrin is deposited (normal) | Abnormal increase in DIC
85
What is therapeutic fibrinolysis?
Induced by injection of direct plasminogen activators
86
What is impaired fibrinolysis?
Due to decreased production, increased destruction, or increased inhibition of plasminogen, plasminogen activators, or plasmin
87
Fibrinogen degradation product test and D-dimer test (fibrin degradation products) in primary fibrinolysis:
FDP: pos | D-dimer: neg
88
Fibrinogen degradation product test and D-dimer test (fibrin degradation products) in secondary fibrinolysis:
FDP: pos | D-dimer: pos
89
Thrombosis is:
abnormal formation in circulation of solid, localized masses of fibrin and/or platelets that cause partial or complete vessel blockage
90
Thromboembolism is:
thrombi break away and block smaller vessels
91
3 factors involved in forming a thrombus are:
Slowed blood flow Hypercoagulability Vessel wall damage
92
Inherited disorders that promote thrombosis include:
``` Factor V Leiden gene mutation (protein C resistance) Antithrombin III deficiency Protein C or S deficiency Homocysteinuria Dysfibrinogenemia Abnormal plasminogen Prothrombin G20210A variant ```
93
What is Factor V Leiden gene mutation (protein C resistance)?
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
What is antithrombin III deficiency?
Normally works with heparin to inhibit factors | Inadequate inhibition of thrombin, Xa, IXa
95
What is Protein C or S deficiency?
Inadequate inhibition of V and VIII | Impaired activation of fibrinolysis because activated protein C inhibits antiplasmin
96
What is homocysteinuria?
Damaged endothelium by deposits of amino acids causes activation of coagulation and atheroschlerosis
97
What is dysfibrinogenemia?
Abnormal fibrinogen makes abnormal fibrin that will not neutralize thrombin
98
What is abnormal plasminogen?
Impaired formation of plasmin
99
Prothrombin G20210A variant
Abnormal allele of factor II = increased FII levels = increased thrombosis
100
What immune condition is an acquired coagulation deficiency and also and acquired disorder that promotes thrombosis?
Lupus anticoagulant
101
Lupus anticoagulant
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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