The antiphospholipid syndrome: still an enigma
The antiphospholipid syndrome: still an enigma
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DOI：
10.1182/asheducation-2015.1.53
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发表时间：
2015-12-01
影响因子：
3
通讯作者：
McCrae, Keith R.
中科院分区：
文献类型：
--
作者：
Chaturvedi, Shruti;McCrae, Keith R.
抗磷脂综合征（APS）的定义是抗磷脂抗体（aPL）患者的临床表现，包括血栓形成和/或胎儿丢失或妊娠发病率。抗磷脂抗体是获得性血栓形成倾向最常见的原因之一，但与大多数遗传性血栓形成倾向不同，它与静脉和动脉血栓形成有关。尽管对aPL进行了大量的临床和基础研究，但尚未定义解释其促血栓形成活性的统一机制;这可能反映了aPL的异质性和/或它们可能影响多种促血栓形成和/或抗血栓形成途径的事实。抗磷脂抗体主要针对磷脂结合蛋白，而不是磷脂本身，最常见的抗原靶标是β 2-糖蛋白1（β 2GPI），尽管针对其他靶标（如凝血酶原）的抗体也有很好的描述。aPL的实验室诊断取决于狼疮抗凝剂（LA）的检测，这是磷脂依赖性抗凝试验的基础，和/或抗心磷脂和抗β 2-糖蛋白1抗体。尽管可能改善结局的新策略正在出现，但无限期抗凝仍然是血栓性APS的主要治疗方法。初步报告提示APS相关血栓形成患者应谨慎使用直接口服抗凝剂。基于有限的证据，阿司匹林和低分子量肝素被推荐用于产科APS。仍然迫切需要更好地了解人类APS的发病机制，确定临床和实验室参数，定义患者的APS相关事件的最大风险，并有针对性地治疗这种常见但神秘的疾病。
Antiphospholipid syndrome (APS) is defined by clinical manifestations that include thrombosis and/or fetal loss or pregnancy morbidity in patients with antiphospholipid antibodies (aPL). Antiphospholipid antibodies are among the most common causes of acquired thrombophilia, but unlike most of the genetic thrombophilias are associated with both venous and arterial thrombosis. Despite an abundance of clinical and basic research on aPL, a unified mechanism that explains their prothrombotic activity has not been defined; this may reflect the heterogeneity of aPL and/or the fact that they may influence multiple pro-and/or antithrombotic pathways. Antiphospholipid antibodies are directed primarily toward phospholipid binding proteins rather than phospholipid per se, with the most common antigenic target being beta 2-glycoprotein 1 (beta 2GPI) although antibodies against other targets such as prothrombin are well described. Laboratory diagnosis of aPL depends upon the detection of a lupus anticoagulant (LA), which prolongs phospholipid-dependent anticoagulation tests, and/or anticardiolipin and anti-beta 2-glycoprotein 1 antibodies. Indefinite anticoagulation remains the mainstay of therapy for thrombotic APS, although new strategies that may improve outcomes are emerging. Preliminary reports suggest caution in the use of direct oral anticoagulants in patients with APS-associated thrombosis. Based on somewhat limited evidence, aspirin and low molecular weight heparin are recommended for obstetrical APS. There remains a pressing need for better understanding of the pathogenesis of APS in humans, for identification of clinical and laboratory parameters that define patients at greatest risk for APS-related events, and for targeted treatment of this common yet enigmatic disorder.