Catastrophic antiphospholipid syndrome.
In its classic presentation, the antiphospholipid syndrome manifests a combination of venous or arterial thrombosis and fetal loss, accompanied by elevations of antibodies directed toward negatively charged phospholipids, as measured by anticardiolipin antibody assays and/or positive lupus anticoagulant tests. The manifestations often include a moderate thrombocytopenia and, less commonly, hemolysis. In contrast, a less frequently encountered subset of the antiphospholipid syndrome, termed the "catastrophic" antiphospholipid syndrome, affects mainly small vessels predominantly supplying organs. The thrombocytopenia is usually marked, and a Coombs positive microangiopathic-type anemia may accompany the condition. Features of disseminated intravascular coagulation may be evident in some patients. It is fatal in approximately 50% of cases reported. Treatment should include not only adequate anticoagulation with intravenous heparin but also full doses of intravenous corticosteroids, to offset the systemic inflammatory response syndrome that occurs as a result of the extensive tissue damage, and plasmapheresis, using fresh frozen plasma. Parenteral antibiotics should be administered early if infection is suspected.
Asherson
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RA
Department of Medicine, The Groote School Hospital, University of Cape Town School of Medicine, South Africa. ashspot@icon.co.za
Cervera
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D016736:Antiphospholipid Syndrome / Q000150:complications / Q000209:etiology* / Q000628:therapy; D002388:Catastrophic Illness; D006801:Humans; D008833:Microcirculation; D009102:Multiple Organ Failure / Q000097:blood / Q000209:etiology / Q000473:pathology; D013927:Thrombosis / Q000209:etiology