Cardiology Clinic, Thrombosis Centre, Department of Cardiac Thoracic and Vascular Sciences, University of Padua, Italy.
Cardiology Clinic, Thrombosis Centre, Department of Cardiac Thoracic and Vascular Sciences, University of Padua, Italy.
Thromb Res. 2018 Sep;169:35-40. doi: 10.1016/j.thromres.2018.07.011. Epub 2018 Jul 7.
Antiphospholipid Syndrome (APS) is a condition characterized by the occurrence of thromboembolic events and/or pregnancy loss combined with one laboratory criterion among Lupus Anticoagulant- LAC, anticardiolipin -aCL, and anti β2-Glycoprotein I -aβ2GPI antibodies. Several hypotheses were put forward to explain the causal role of antibodies in the clinical events but none is fully convincing. Current laboratory diagnosis is based on three tests (LAC, IgG/IgM aβ2GPI and IgG/IgM aCL antibodies). The triple-positive profile (all the three tests positive, same isotype) is associated with a higher risk for thrombosis. The mainstay of therapy in thrombotic APS is anticoagulation, with VKAs being the cornerstone. Low dose aspirin in combination or alone may have a role in arterial thrombosis, and in primary thromboprophylaxis. The Non-Vitamin K Antagonists Oral Anticoagulants (NOACs) role in the therapy of APS is under investigation but not verified. Alternative treatment options including rituximab and eculizumab have been successfully reported in few cases of catastrophic APS.
抗磷脂综合征（APS）是一种以血栓栓塞事件和/或妊娠丢失为特征的疾病，同时伴有狼疮抗凝物（LAC）、抗心磷脂抗体（aCL）和抗β2-糖蛋白 I 抗体（aβ2GPI）中的一项实验室标准。人们提出了几种假设来解释抗体在临床事件中的因果作用，但没有一种是完全令人信服的。目前的实验室诊断基于三项检测（LAC、IgG/IgM aβ2GPI 和 IgG/IgM aCL 抗体）。三阳性（三种检测均阳性，同种同型）与血栓形成风险增加相关。血栓性 APS 的主要治疗方法是抗凝治疗，维生素 K 拮抗剂（VKA）是基石。低剂量阿司匹林联合或单独应用可能对动脉血栓形成和原发性血栓预防有作用。新型口服抗凝药物（NOACs）在 APS 治疗中的作用正在研究中，但尚未得到证实。利妥昔单抗和依库珠单抗等替代治疗方案已在少数灾难性 APS 病例中成功报告。
