Tohidi-Esfahani Ibrahim, Mittal Prabal, Isenberg David, Cohen Hannah, Efthymiou Maria
Haematology Department, Concord Repatriation General Hospital, Sydney, NSW 2139, Australia.
Sydney Medical School, Faculty of Medicine and Health, University of Sydney, Sydney, NSW 2050, Australia.
J Clin Med. 2024 Jan 27;13(3):741. doi: 10.3390/jcm13030741.
Antiphospholipid antibody syndrome (APS) is an autoimmune disorder characterised by thrombosis and the presence of antiphospholipid antibodies (aPL): lupus anticoagulant and/or IgG/IgM anti-β2-glycoprotein I and anticardiolipin antibodies. APS carries significant morbidity for a relatively young patient population from recurrent thrombosis in any vascular bed (arterial, venous, or microvascular), often despite current standard of care, which is anticoagulation with vitamin K antagonists (VKA). Platelets have established roles in thrombosis at any site, and platelet hyperreactivity is clearly demonstrated in the pathophysiology of APS. Together with excess thrombin generation, platelet activation and aggregation are the common end result of all the pathophysiological pathways leading to thrombosis in APS. However, antiplatelet therapies play little role in APS, reserved as a possible option of low dose aspirin in addition to VKA in arterial or refractory thrombosis. This review outlines the current evidence and mechanisms for excessive platelet activation in APS, how it plays a central role in APS-related thrombosis, what evidence for antiplatelets is available in clinical outcomes studies, and potential future avenues to define how to target platelet hyperreactivity better with minimal impact on haemostasis.
抗磷脂抗体综合征（APS）是一种自身免疫性疾病，其特征为血栓形成以及抗磷脂抗体（aPL）的存在：狼疮抗凝物和/或IgG/IgM抗β2糖蛋白I抗体及抗心磷脂抗体。对于相对年轻的患者群体而言，APS会因任何血管床（动脉、静脉或微血管）反复出现血栓而导致显著的发病率，即便采用当前的标准治疗方法（即使用维生素K拮抗剂（VKA）进行抗凝治疗）亦是如此。血小板在任何部位的血栓形成过程中都发挥着既定作用，并且在APS的病理生理学中明显表现出血小板反应过度。与凝血酶生成过多一起，血小板激活和聚集是导致APS血栓形成的所有病理生理途径的共同最终结果。然而，抗血小板治疗在APS中作用甚微，仅作为在动脉血栓形成或难治性血栓形成中除VKA之外加用低剂量阿司匹林的一种可能选择。本综述概述了目前关于APS中血小板过度激活的证据和机制、其在APS相关血栓形成中如何发挥核心作用、临床结局研究中抗血小板治疗的可用证据，以及未来确定如何以对止血影响最小的方式更好地靶向血小板反应过度的潜在途径。
