True about anti-cardiolipin antibody syndrome among the following is/are
Correct Answer: All of the above
Description: Ans. d (All of the above ). (Ref. Harrison, Medicine, 18th/Ch. 320) ANTIPHOSPHOLIPID Syndrome (APS)/ANTICARDIOLIPIN ANTIBODY SYNDROME^ Definition: # APS is an autoantibody-mediated acquired thrombophilia characterized by recurrent arterial or venous thrombosis and/or pregnancy morbidity in the presence of autoantibodies against phospholipid (PL)-binding plasma proteins, mainly a plasma apolipoprotein known as 2 glycoprotein I (2GPI) and prothrombin. Another group of antibodies termed lupus anticoagulant (LA) prolong clotting times in vitro; this prolongation is not corrected by adding normal plasma to the detection system. APS may occur alone (primary), or in association with any other autoimmune disease (secondary). Catastrophic APS (CAPS) is defined as a rapidly progressive thromboembolic disease involving simultaneously three or more organs, organ systems, or tissues leading to corresponding functional defects. # The antiphospholipid antibody is IgA or IgM against enzymes in the coagulation cascade. Antibodies bind to phospholipids in vitro and prolongs PTT. Antibody predisposes to thrombosis by maintaining platelets in inactivated state or by inhibiting the fibrinolytic activity of endothelial cells. Bleeding manifests in presence of thrombocytopenia hypoprothrombinaemia. Features: # Recurrent fetal loss (mid-trimester abortions) # Recurrent thrombotic events # Thrombocytopenia/Pancytopenia # IUGR and placental abruption # Pregnancy-induced hypertension (PIH) # Diagnostic test : is Russel viper venom PTT. Prolongation of Russel viper venom time is more sensitive, it is neutralized by adding platelet membrane (lupus anticoagulant). # Treatment--After the first thrombotic event, APS patients should be placed on warfarin for life aiming to achieve an INR of 2.5 to 3.5, alone or in combination with 80 mg of aspirin daily. Pregnancy morbidity is prevented by a combination of heparin with aspirin 80 mg daily. IVIg 400 mg/kg qd for 5 days may also prevent abortions, while glucocorticoids are ineffective. Aspirin 80 mg daily protects patients with SLE positive for aPL antibodies from developing thrombotic events.
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