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14 Cards in this Set

  • Front
  • Back
Congenital
1.) Deficiencies
2.) Excesses
3.) Mutations
Deficiencies
1.) ATIII
2.) Protein C
3.) Protein S
Excesses
1.) plasminogen activator inhibitor
2.) other inhibitors of anticoagulants
3.) clotting factors, plts, fibrinogen, vWF
Plasminogen activator inhibitor
stops plasminogen to break down clots and allow excess clot to stay
Mutations
1.) Loss of function: ATIII, PC, PS (qualitative defect of anticoagulants)
2.) Gain of function: Factor V leiden, prothrombin 20210A
Factor V leiden
- 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)
Prothrombin 20210A
substitution of lysine and also adenine for guanine in the gene leading to increased levels of prothrombin
Acquired
1.) Myeloproliferative disorders
2.) Autoantibodies
3.) Diabetes
4.) Lupus
5.) Pregnancy
6.) Cancer
Cancer and thrombosis
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
Testing for hypercoagulation
FDP, D-dimer, Thrombin/antithrombin complexes, specific markers of activation
Heparin induced thrombocytopenia (HITT)
have this antibody, risk of thrombosis
Anti-phospholipid syndrome
- most common cause of thrombosis
1.) Lupus anticoagulant
2.) anti-cardiolipin antibodies
Anti-cardiolipin antibodies
- 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
Specific inhibitors
- 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