2.1 Thrombophilia Flashcards

1
Q

What are the three components of thrombus formation?

A

Hypercoaguability
Abnormal blood flow
Endothelial damage

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2
Q

What are the genetic risk factors for vein thrombosis?

A

AT deficiency. PC and PS deficiency, FV Leiden, APCR, ProThrombin G20210A, MTHFR mutation, increased VIIIc

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3
Q

What are the acquired risk factors for vein thrombosis?

A

Cancer, surgery, acute illness with bed rest

Pregnancy/puerperium, hormonal preparations (OCP), obesity, autoimmune disease, elevated homocysteine

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4
Q

Define thrombophilia

A

abnormal occurrence of vein thrombosis without adequate provocation

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5
Q

Define thrombophilia

A

abnormal occurrence of vein thrombosis without adequate provocation

abnormal = young patients, unprovoked, recurrent, unusual sites, family history

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6
Q

What are the coagulation factors?

A
I: Fibrinogen 
II: Prothrombin 
III: Tissue Factor or Thromboplastin 
IV: Ca 
V: Labile factor 
VII: Proconvertin 
VIII: Antihemophilic factor
IX: Christmas factor
X: Stuart or Stuart-Prower factor 
XI: Plasma thromboplastin antecedent 
XII: Hageman factor, contact factor 
XIII: Fibrin stablizing factor 

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7
Q

What are the genetic risk factors for vein thrombosis?

A
AT deficiency
Protein C and Protein S deficiency
FV Leiden
APCR
ProThrombin G20210A, MTHFR mutation
Increased VIIIc
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8
Q

What are the coagulation factors?

A
I: Fibrinogen 
II: Prothrombin 
III: Tissue Factor or Thromboplastin 
IV: Ca 
V: Labile factor 
VII: Proconvertin 
VIII: Antihemophilic factor
IX: Christmas factor
X: Stuart or Stuart-Prower factor 
XI: Plasma thromboplastin antecedent 
XII: Hageman factor, contact factor 
XIII: Fibrin stablizing factor 

Freshers Party Tonight Come Lets Play And Call Sarah Please Have Fun

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9
Q

What is fibrinolysis?

A

The breakdown of clots from plasmin (fibrin degradation products)

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10
Q

What factors promote the production of plasmin from plasminogen?

A

tPA (from endothelial cells) and Urokinase Streptokinase

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11
Q

What is the clinical definition of thrombophilia?

A

Patients who develop PE spontaneously, VTE is out of proportion to any recognised stimulus, recurrent venous thrombosis and developing venous thromboembolism at an early age

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12
Q

What patients have been shown to have high rates of VTE?

A

Cancer patients (20% new cases associated with cancer) - especially those with pancreatic and stomach cancer

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13
Q

What is antiphospholipid syndrome?

A

A non inflammatory auto immune disease in which there is presence of antibodies against anionic phospholipids. Associated with the occurrence of venous and arterial thrombosis and/or recurrent miscarriage.

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14
Q

What are the most common antibodies directed against in antiphospholipid syndrome?

A

B2 glycoprotein 1 and prothrombin

anti B2 glycoprotein 1 antibodies

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15
Q

What do the antiphospholipid antibodies do?

A

Inhibit the inhibiting factors of the coagulation cascade.

Interfere with coagulation and anticoagulation pathways and activation of platelets.

Endothelial activation in vessels

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16
Q

Why does deficient protein C usually occur?

A

resistance of factor Va to be cleaved by activated protein C leading to excessive levels of factor V stimulating clotting

Usually because of Factor V Leiden mutation

17
Q

What mutation leads to elevated prothrombin?

A

mutation at position 20210

18
Q

What is the role of antithrombin?

A

form a 1:1 complex with activated clotting factors (thrombin and Xa) at the active site thus preventing the protease enzymes that would normally bind from doing so.

19
Q

When does antithrombin accelerate its action?

A

In the presence of heparin or when it is activated by cell surface heparin sulfate

20
Q

What two factors does protein C inhibit?

A

factor Va and VIIIa

21
Q

What is the role of protein S?

A

to act as a cofactor for protein C in the inactivation of FVa and FVIIIa

22
Q

When do you usually test for thrombophilia?

A

1 month after commencing therapy

23
Q

When do you usually test for thrombophilia?

A

1 month after completion therapy

24
Q

Who do you normally test for thrombophilia?

A

Recurrent/spontaneous PE or DVT
Thrombosis at young age
Family history or thrombosis at unusual site

25
Q

What tests do you use to evaluate thrombophilia

A
FBC, FCP (clotting) and ESR 
Factor V leiden (ACPR) 
Prothrombin mutation 
AT 
Protein C and S 
Lupus anticoagulant
26
Q

Which two abnormalaties will cause both arterial and venous clotting?

A

Hyperchromocysteinemia and Lupus anticoagulant