Robertson Beverley, Greaves Mike
Department of Haematology, Aberdeen Royal Infirmary, ABERDEEN, AB25 2ZN, Scotland, UK.
Blood Rev. 2006 Jul;20(4):201-12. doi: 10.1016/j.blre.2005.12.001. Epub 2006 Jan 27.
Antiphospholipid Syndrome (APS) is a form of immune mediated thrombophilia, presenting as recurrent thrombotic events and pregnancy morbidity, in association with positive laboratory tests for antiphospholipid antibodies (APA) in the form of Lupus Anticoagulant (LA) or anticardiolipin antibodies(ACA). Insights into the pathophysiology of the condition suggest that some antibodies are prothrombotic in vivo, and that the mechanism of thrombosis is likely to be multifactorial. APS has a broad spectrum of clinical presentations, and the laboratory diagnosis can be difficult due to heterogeneity of APAs and poor standardisation of laboratory tests. Anticoagulation is the mainstay of the management of the thrombotic and obstetric complications of APS. The risk of recurrent thrombosis appears to be high, and the duration and intensity of therapy remains controversial. Randomised controlled trials have shown that standard intensity anticoagulation is adequate in most cases of venous thrombosis. Further trials are required to establish whether high intensity coagulation is of benefit in recurrent or arterial thrombosis. The optimal management of recurrent fetal loss is debated and large studies are required to establish a clear benefit of heparin and aspirin over aspirin alone or supportive care.
抗磷脂综合征(APS)是一种免疫介导的血栓形成倾向疾病,表现为反复出现血栓事件和妊娠并发症,同时实验室检查抗磷脂抗体(APA)呈阳性,包括狼疮抗凝物(LA)或抗心磷脂抗体(ACA)。对该疾病病理生理学的深入研究表明,某些抗体在体内具有促血栓形成作用,且血栓形成机制可能是多因素的。APS临床表现多样,由于APA的异质性和实验室检测标准化程度低,实验室诊断可能存在困难。抗凝治疗是APS血栓形成和产科并发症管理的主要手段。复发性血栓形成的风险似乎很高,治疗的持续时间和强度仍存在争议。随机对照试验表明,标准强度抗凝在大多数静脉血栓形成病例中是足够的。需要进一步试验来确定高强度抗凝在复发性或动脉血栓形成中是否有益。复发性胎儿丢失的最佳管理方法存在争议,需要大型研究来确定肝素和阿司匹林联合使用相对于单独使用阿司匹林或支持性治疗是否具有明显优势。