Bertolaccini Maria Laura, Hughes Graham Rv, Khamashta Munther A
The Rayne Institute, St Thomas' Hospital, London, UK.
Clin Lab. 2004;50(11-12):653-65.
The antiphospholipid syndrome (APS) is a multi-system prothrombotic disorder associated with circulating auto-antibodies directed against various phospholipid-binding proteins. The major clinical manifestations are recurrent arterial or venous thrombosis, but due to its heterogeneity, atypical presentations can obscure the diagnosis. Decisions regarding when to attribute complications to aPL are difficult. The most established tests are lupus anticoagulant (LA) detected by clotting assays and anticardiolipin (aCL) detected by ELISA. Although LA and aCL assays are clinically useful, these tests do not clearly differentiate antibodies with different specificities. Antibodies to beta2GPI are associated with thrombosis in the APS. Although these antibodies are detected by aCL assay (e.g. beta2GPI-dependent aCL), some aCL are not associated with the syndrome (e.g. beta2GPI-independent aCL). Regarding LAs, more studies are needed to determine if it is clinically important to differentiate specificities against beta2GPI or prothrombin. The role of aPLs in the pathogenesis of thrombosis requires further and intensive investigation. If autoantibodies to particular phospholipid binding proteins are shown to be associated with different clinical presentations or to confer different risks, the availability of more accurate diagnostic techniques will be required for the recognition of pathogenic aPLs. By now, clinical judgement, careful exclusion of other etiologies and serial aPL levels are helpful in this regard.
抗磷脂综合征（APS）是一种多系统血栓形成前状态的疾病，与针对各种磷脂结合蛋白的循环自身抗体有关。主要临床表现为反复发生的动脉或静脉血栓形成，但由于其异质性，非典型表现可能会掩盖诊断。判断何时将并发症归因于抗磷脂抗体（aPL）很困难。最常用的检测方法是通过凝血试验检测狼疮抗凝物（LA）和通过酶联免疫吸附测定（ELISA）检测抗心磷脂抗体（aCL）。尽管LA和aCL检测在临床上有用，但这些检测并不能清楚地区分具有不同特异性的抗体。抗β2糖蛋白I（β2GPI）抗体与APS中的血栓形成有关。尽管这些抗体可通过aCL检测（例如β2GPI依赖性aCL）检测到，但一些aCL与该综合征无关（例如β2GPI非依赖性aCL）。关于LA，需要更多的研究来确定区分针对β2GPI或凝血酶原的特异性在临床上是否重要。aPL在血栓形成发病机制中的作用需要进一步深入研究。如果针对特定磷脂结合蛋白的自身抗体被证明与不同的临床表现相关或具有不同的风险，则需要更准确的诊断技术来识别致病性aPL。到目前为止，临床判断、仔细排除其他病因以及连续检测aPL水平在这方面是有帮助的。
