Coagulation Laboratory, Ghent University Hospital, Department of Diagnostic Sciences, Ghent University, Ghent, Belgium.
Int J Lab Hematol. 2024 May;46 Suppl 1:34-42. doi: 10.1111/ijlh.14268. Epub 2024 Apr 7.
Antiphospholipid syndrome (APS) is an autoimmune disease characterized by thrombotic manifestations and/or obstetric complications in patients with persistently positive antiphospholipid antibodies (aPL). aPL are a heterogeneous group of autoantibodies, but only lupus anticoagulant, anticardiolipin (aCL), and antibeta2-glycoprotein I antibodies (aβ2GPI) IgG or IgM are included as laboratory classification criteria. Seronegative APS patients are usually defined as patients with the clinical symptoms of APS but who test negative for aPL. The negativity to classic aPL criteria does not exclude the presence of other aPL. Several noncriteria aPL have been identified. Some noncriteria aPL are well studied, such as IgA aCL and aβ2GPI, the antiphosphatidylserine-prothrombin (aPS/PT) antibodies, and the antibodies against the domain I of beta2-glycoprotein I (aDI), both latter groups receiving more attention for their role in thrombotic events and pregnancy complications. Other noncriteria aPL that have been studied are antibodies against annexin V, prothrombin, phosphatidylethanolamine, phosphatidic acid, phosphatidylserine, phosphatidylinositol, vimentin-cardiolipin complex, anti-protein S/protein C. Measurement of some of these noncriteria aPL (aPS/PT, aDI) is useful in the laboratory work-out of APS in specific situations. We have to differentiate between patients who are positive for noncriteria aPL only, and patients who have both criteria and noncriteria aPL to enable us to study their role in the diagnosis or risk stratification of APS. The research on noncriteria aPL is continually developing as the clinical relevance of these antibodies is not yet fully clarified.
抗磷脂综合征(APS)是一种自身免疫性疾病,其特征为持续存在抗磷脂抗体(aPL)的患者出现血栓表现和/或产科并发症。aPL 是一组异质性自身抗体,但只有狼疮抗凝物、抗心磷脂(aCL)和抗β2-糖蛋白 I 抗体(aβ2GPI)IgG 或 IgM 被纳入实验室分类标准。血清阴性 APS 患者通常定义为具有 APS 临床症状但 aPL 检测为阴性的患者。对经典 aPL 标准的阴性并不能排除其他 aPL 的存在。已经鉴定出几种非标准 aPL。一些非标准 aPL 已经得到了很好的研究,例如 IgA aCL 和 aβ2GPI、抗磷脂酰丝氨酸-凝血酶原(aPS/PT)抗体和抗β2-糖蛋白 I 结构域 I(aDI)的抗体,这后两组因其在血栓形成事件和妊娠并发症中的作用而受到更多关注。其他已研究过的非标准 aPL 包括抗 annexin V、凝血酶原、磷脂酰乙醇胺、磷脂酸、磷脂酰丝氨酸、磷脂酰肌醇、波形蛋白-心磷脂复合物、抗蛋白 S/蛋白 C 的抗体。在特定情况下,测量这些非标准 aPL(aPS/PT、aDI)中的一些有助于进行 APS 的实验室诊断。我们必须区分仅为非标准 aPL 阳性的患者和同时具有标准和非标准 aPL 的患者,以便能够研究它们在 APS 诊断或风险分层中的作用。由于这些抗体的临床相关性尚未完全阐明,因此非标准 aPL 的研究仍在不断发展。