Catastrophic antiphospholipid syndrome

被引:28
作者
Asherson, RA [1 ]
Cervera, R
机构
[1] Univ Cape Town, Sch Med, Groote Sch Hosp, Dept Med,Rheumat Dis Unit, ZA-17937 Cape Town, South Africa
[2] Hosp Clin Barcelona, System Autoimmune Dis Unit, Barcelona, Catalonia, Spain
关键词
D O I
10.1097/00062752-200009000-00012
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
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 plasma pheresis, using fresh frozen plasma. Parenteral antibiotics should be administered early if infection is suspected. (C) 2000 Lippincott Williams & Wilkins, Inc.
引用
收藏
页码:325 / 329
页数:5
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