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14 Cards in this Set
- Front
- Back
Congenital
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1.) Deficiencies
2.) Excesses 3.) Mutations |
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Deficiencies
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1.) ATIII
2.) Protein C 3.) Protein S |
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Excesses
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1.) plasminogen activator inhibitor
2.) other inhibitors of anticoagulants 3.) clotting factors, plts, fibrinogen, vWF |
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Plasminogen activator inhibitor
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stops plasminogen to break down clots and allow excess clot to stay
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Mutations
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1.) Loss of function: ATIII, PC, PS (qualitative defect of anticoagulants)
2.) Gain of function: Factor V leiden, prothrombin 20210A |
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Factor V leiden
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- adenine substitution for guanine at position 506 gaining a resistance to inactivation by protein
- Cleaving site for aPC is changed causing resistance to inactivation - Resulting in an enhanced FV functionality (thrombosis) |
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Prothrombin 20210A
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substitution of lysine and also adenine for guanine in the gene leading to increased levels of prothrombin
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Acquired
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1.) Myeloproliferative disorders
2.) Autoantibodies 3.) Diabetes 4.) Lupus 5.) Pregnancy 6.) Cancer |
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Cancer and thrombosis
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1.) tumor cells express and release procoagulants like tissue factor
2.) decrease in normal anticoagulants 3.) Acquired APC resistance (not FV leiden) 4.) impaired fibrinolysis |
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Testing for hypercoagulation
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FDP, D-dimer, Thrombin/antithrombin complexes, specific markers of activation
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Heparin induced thrombocytopenia (HITT)
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have this antibody, risk of thrombosis
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Anti-phospholipid syndrome
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- most common cause of thrombosis
1.) Lupus anticoagulant 2.) anti-cardiolipin antibodies |
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Anti-cardiolipin antibodies
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- PT high and PTT high in 40-50%
- high frequency in AIDs - 53% critical pts develop Lupus anticoagulant after 13 days but 63% of those become negative after 17 days - Test using dRVVT, ELISA * snake venoms are not inhibited by heparin and are used as thrombin-like enzymes in testing - RRV: used for factor V, VII, X, and LE-A which is followed by neutralization to confirm |
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Specific inhibitors
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- factor VIII and IX
- secondary to treatment - change source of factor, porcine (pig), recombinant - immune tolerance therapy, pheresis, or immunoadsorption to reduce levels and months of high dose therapy infusion * 50-75% success |