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[抗磷脂综合征,最新进展]

[The antiphospholipid syndrome, an update].

作者信息

Alonso Santor J E, Inglada Galiana L, Pérez Paredes G

机构信息

Servicio de Medicina Interna, Hospital Universitario Río Hortega, Valladolid, Spain.

出版信息

An Med Interna. 2007 May;24(5):242-8. doi: 10.4321/s0212-71992007000500009.

DOI:10.4321/s0212-71992007000500009
PMID:17907891
Abstract

The antiphospholipid syndrome is an antibody mediated hypercoagulable state characterized by recurrent venous and arterial thromboembolic events. Several studies have determined that the frequency of antiphospholipid syndrome in patients presenting with a venous thromboembolic event is between 4% and 14%. Classical criteria include the presence of anticardiolipin antibody or lupus anticoagulant with typical complications of thrombosis or pregnancy loss. Other common associated manifestations include livedo reticularis, thrombocytopenia, valvular heart disease, and nephropathy with renal insufficiency, hypertension and proteinuria. Because of the high risk for recurrent thromboembolism in these patients, current recommendations suggest a longer, potentially lifelong, course of antithrombotic therapy following an initial event. For an initial venous thromboembolic event, a target INR of 2.0 to 3.0 is supported by two prospective, randomized clinical trials. In contrast, relatively limited data exist for an initial arterial thromboembolic event in patients who have the antiphospholipid syndrome, and therapeutic recommendations range from aspirin to warfarin with a high target INR. Recurrent thromboembolic events can be extremely difficult to treat, and some patients may benefit from the addition of immunosuppressive therapies. It is very important to evaluate in this setting additional, coincident prothrombotic risk factors.

摘要

抗磷脂综合征是一种抗体介导的高凝状态,其特征为反复发生静脉和动脉血栓栓塞事件。多项研究已确定,出现静脉血栓栓塞事件的患者中抗磷脂综合征的发生率在4%至14%之间。经典标准包括存在抗心磷脂抗体或狼疮抗凝物以及典型的血栓形成或流产并发症。其他常见的相关表现包括网状青斑、血小板减少、瓣膜性心脏病以及伴有肾功能不全、高血压和蛋白尿的肾病。由于这些患者反复发生血栓栓塞的风险很高,目前的建议是在首次事件后进行更长疗程、可能是终身的抗栓治疗。对于首次静脉血栓栓塞事件,两项前瞻性随机临床试验支持的目标国际标准化比值(INR)为2.0至3.0。相比之下,关于抗磷脂综合征患者首次动脉血栓栓塞事件的数据相对有限,治疗建议范围从阿司匹林到高目标INR的华法林。反复发生的血栓栓塞事件可能极难治疗,一些患者可能会从加用免疫抑制疗法中获益。在这种情况下评估其他同时存在的促血栓形成危险因素非常重要。

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