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14 Cards in this Set
- Front
- Back
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causes of congenital thrombophilia
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deficiency of anticoagulants: ATIII, Protein C, Protein S
Resistance to cofactor proteolysis: factor V leiden High levels of procoagulants: prothrombin 20210 mutation damage to endothelium: homocysteinemia |
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what anticoagulant binds to ATIII
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heparin
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what does ATIII inhibit?
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vitamin k dependent factors - especially 2 and 10
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what activates protein C?
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thrombin bound to thrombomodulin on endothelium
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What does activated protein C
(APC) do? |
cleaves FVa and FVIIIa
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what is different about protein S compared to protein C and ATIII
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protein S is a cofactor not an enzyme
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where is ATII, Protein C and Protein S made?
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ATIII: liver
Protein C: liver Protein S: liver, endothelium, platelets |
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how does protein S circulate
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free and bound to C4b binding protein
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why does FV Leiden cause thrombosis?
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normally the breakdown products of FV are anticoagulants but FV Leiden b/c of mutation FV does not get broken down by activated protein C
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what is the risk for thrombosis in heterozygous/homozygous FV Leiden
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heterozygous: 10 fold increase risk
homozygous: 50-100 fold increase |
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what is the functional result of the prothrombin G20210A mutation?
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20% increase in prothrombin (get increased levels of prothrombin mRNA)
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what causes antiphospholipid syndrome (names)
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lupus anticoagulant
anticardiolipin anti-beta 2 glycoprotein 1 |
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what are the confirmatory tests for lupus anticoagulant?
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russell's viper venom test (RVVT)
as well as correction by excess phospholipid |
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what predisposes to ARTERIAL thrombosis?
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antiphospholipid syndrome
homocysteinemia/homocystinuria Umbilical artery line |
