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14 Cards in this Set

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