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狼疮抗凝物/抗磷脂综合征

The lupus anticoagulant/antiphospholipid syndrome.

作者信息

Shapiro S S

机构信息

Cardeza Foundation for Hematologic Research, Department of Medicine, Jefferson Medical College of Thomas Jefferson University, Philadelphia, Pennsylvania 19107-5099, USA.

出版信息

Annu Rev Med. 1996;47:533-53. doi: 10.1146/annurev.med.47.1.533.

DOI:10.1146/annurev.med.47.1.533
PMID:8712801
Abstract

Lupus anticoagulants and anticardiolipin antibodies have been strongly associated with the risk of thrombosis, recurrent fetal loss, thrombocytopenia, and a number of other clinical manifestations that together have been referred to as the antiphospholipid syndrome. Despite growing evidence of the significance of this relationship, the pathogenetic mechanisms involved are largely unknown. Recent data suggest strongly that lupus anticoagulants (LACs) and anticardiolipin antibodies (ACAs) are antibodies to protein-phospholipid complexes rather than to phospholipids, as had originally been thought, and that other protein-phospholipid complexes, not recognized by standard assays for LACs or ACAs, may also exist in patients with the antiphospholipid syndrome. Although very recent experimental data may lead to new therapeutic approaches in this syndrome, at present we can only deal with the thrombotic risk by the use of long-term anticoagulation. This chapter reviews current methods of diagnosis, concepts of pathogenesis, and the basis for an approach to anticoagulation in patients at risk for thrombosis or other manifestations of the antiphospholipid syndrome.

摘要

狼疮抗凝物和抗心磷脂抗体与血栓形成风险、复发性流产、血小板减少症以及许多其他临床表现密切相关,这些临床表现统称为抗磷脂综合征。尽管越来越多的证据表明这种关系的重要性,但其中涉及的发病机制在很大程度上仍不清楚。最近的数据有力地表明,狼疮抗凝物(LACs)和抗心磷脂抗体(ACAs)是针对蛋白质 - 磷脂复合物的抗体,而非如最初所认为的针对磷脂,并且抗磷脂综合征患者中可能还存在其他未被LACs或ACAs标准检测方法识别的蛋白质 - 磷脂复合物。尽管最近的实验数据可能会为该综合征带来新的治疗方法,但目前我们只能通过长期抗凝来应对血栓形成风险。本章综述了当前的诊断方法、发病机制概念以及针对有血栓形成风险或抗磷脂综合征其他表现的患者进行抗凝治疗的依据。

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The lupus anticoagulant/antiphospholipid syndrome.狼疮抗凝物/抗磷脂综合征
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