恶性抗磷脂综合征7例临床分析并文献复习  

Clinical Analysis of 7 Cases With Catastrophic Antiphospholipid Syndrome and Review of Literature

作　　者：曾婷[1] 杨程德[1] 

机构地区：[1]上海交通大学医学院附属仁济医院,上海200001

出　　处：《内科急危重症杂志》2008年第2期63-65,共3页Journal of Critical Care In Internal Medicine

基　　金：国家自然基金项目(No:30772009);上海市教委重点学科建设项目(No:T0203)

摘　　要：目的:探讨恶性抗磷脂综合征(CAPS)的临床特点及预后,以期提高临床医生对该病的认识及诊治水平。方法:回顾性分析我院风湿科收治的7例恶性抗磷脂综合征患者临床表现、实验室检查及预后等资料并复习相关文献。结果:7例CAPS患者中,1例为原发APS,6例为SLE继发APS。静脉血栓发生5例次,主要是下肢深静脉血栓、眼中央静脉栓塞;动脉血栓13例次,以脑梗死为主(6例次)。6例抗心磷脂抗体阳性,3例抗β2-GPⅠ抗体阳性,3例狼疮抗凝物阳性。5例患者在1周内相继出现心、肺、肾等多器官功能衰竭而导致死亡。结论:CAPS患者短时间内发生大量微血栓形成,可导致多器官功能衰竭。本病的预后差,及早诊断、及时治疗是改善患者预后的关键。Objective： To investigate the clinical characteristics and prognosis of catastrophic antiphospholipid syndrome （CAPS）, in order to improve physicain＇s recognization. Methods： The clinical manifestations and laboratory examinations of 7 patients with catastrophic antiphospholipid syndrome were analyzed retrospectively. Results： Of 7 CAPS patients, 1 was primary APS and 6 were secondary to SLE. Venous thrombosis occurred in 5 patients, mostly involved deep veins of lower extremities and central veins of retina. Of 13 arterial thrombosis, cerebral infarction （6 times） was the most commom one. Anticardiolipin antibody was positive in 6 patients, anti-beta-2 glycoprotein I antibody was positive in 3 patients, and lupus anticoagulant was positive in 3 patients. Death due to multiple organ failure within one week was seen in 5 patients. Conclusion： CAPS is characterized by development of extensive microvascular thromboses in multiple organs, occurring simultaneously or within a short period of time and causing multiple organ failure. As CAPS had high mortality, early diagnosis and treatment play key roles for improving the prognosis of CAPS.

关 键 词：恶性 抗磷脂综合征 临床分析 

分 类 号：R593.2[医药卫生—内科学]