Catastrophic antiphospholipid syndrome

In its classic presentation, the antiphospholipid syndrome manifests a comb ination of venous or arterial thrombosis and fetal loss, accompanied by ele vations of antibodies directed toward negatively charged phospholipids, as measured by anticardiolipin antibody assays and/or positive lupus anticoagu lant tests. The manifestations often include a moderate thrombocytopenia an d, less commonly, hemolysis. In contrast, a less frequently encountered sub set of the antiphospholipid syndrome, termed the "catastrophic" antiphospho lipid syndrome, affects mainly small vessels predominantly supplying organs . The thrombocytopenia is usually marked, and a Coombs positive microangiop athic-type anemia may accompany the condition. Features of disseminated int ravascular coagulation may be evident in some patients. It is fatal in appr oximately 50% of cases reported. Treatment should include not only adequate anticoagulation with intravenous heparin but also full doses of intravenou s corticosteroids, to offset the systemic inflammatory response syndrome th at occurs as a result of the extensive tissue damage, and plasma pheresis, using fresh frozen plasma. Parenteral antibiotics should be administered ea rly if infection is suspected. (C) 2000 Lippincott Williams & Wilkins, Inc.