Clinical Chemistry and Hematology, University Medical Center Utrecht, Utrecht, The Netherlands; Sanquin Research, Sanquin Blood Supply Foundation, Amsterdam, The Netherlands.
Thromb Res. 2013 Sep;132(3):319-26. doi: 10.1016/j.thromres.2013.07.023. Epub 2013 Aug 2.
The antiphospholipid syndrome (APS) is diagnosed by the combination of vascular thrombosis and/or pregnancy morbidity and the detection of antiphospholipid antibodies (aPLs) in plasma. In the last few years, a great effort has been made to unravel the mechanism by which aPLs cause thrombosis and a vast amount of mechanisms have been proposed. aPLs were proposed to induce a prothrombotic state by influencing the cellular blood compartment, the plasma compartment, the vascular wall and even metabolic pathways beyond the hemostatic system. However, due to the diversity in the mechanisms and the differences in the methodology, the focus of the mechanistical studies in this field seems to be largely diffused. It is hard to imagine that aPLs can exert such a diversity of effects, resulting in either thrombosis and/or pregnancy morbidity and the relationship between aPLs and the clinical manifestations remains to be a mysterious "black box". In an attempt to get insight in what takes place inside the black box, we have analyzed 126 mechanistical studies on aPLs and discussed differences in the type of antibodies that were used, the involvement of beta2-glycoprotein I (β2GPI), and the criteria used to diagnose APS patients.
抗磷脂综合征（APS）的诊断标准为血管血栓形成和/或妊娠并发症，以及血浆中存在抗磷脂抗体（aPL）。在过去的几年中，人们做出了巨大的努力来揭示 aPL 引起血栓形成的机制，并提出了大量的机制。aPL 被认为通过影响细胞血液成分、血浆成分、血管壁甚至凝血系统以外的代谢途径来诱导血栓形成状态。然而，由于机制的多样性和方法学的差异，该领域的机制研究重点似乎在很大程度上分散了。很难想象 aPL 可以产生如此多样的影响，导致血栓形成和/或妊娠并发症，而 aPL 与临床表现之间的关系仍然是一个神秘的“黑匣子”。为了深入了解黑匣子内部的情况，我们分析了 126 项关于 aPL 的机制研究，并讨论了所使用的抗体类型、β2-糖蛋白 I（β2GPI）的参与以及用于诊断 APS 患者的标准的差异。
