Department of Medicine Solna, Karolinska Institute, Stockholm, Sweden.
Division of Rheumatology, Karolinska University Hospital, Stockholm, Sweden.
Semin Thromb Hemost. 2021 Jul;47(5):506-511. doi: 10.1055/s-0041-1725098. Epub 2021 Apr 20.
The pathogenesis of the antiphospholipid syndrome (APS) is complex and involves the persistent presence of antiphospholipid antibodies (aPL) in the bloodstream causing a prothrombotic condition. aPL induce excessive activation of the endothelium, monocytes, and platelets in consort with aberrations in hemostasis/clotting, fibrinolytic system, and complement activation. Impaired fibrinolysis has been found in APS patients with thrombotic as well as obstetric manifestations. Increased levels of plasminogen activator inhibitor-1 and thrombin-activatable fibrinolysis inhibitor, together with the presence of aPL against annexin-2, tissue-type plasminogen activator, and plasminogen contribute to the compromised fibrinolytic activity in these patients. Furthermore, unfavorably altered fibrin morphology, less amenable to fibrinolysis, has been proposed as a novel prothrombotic mechanism in APS. This review aims to summarize the present knowledge of the mechanisms involved in impaired fibrinolysis in APS patients. We also present a case from clinical practice as an illustration of fibrinolysis impairment in APS patients from a real-life setting.
抗磷脂综合征（APS）的发病机制复杂，涉及血液中持续存在抗磷脂抗体（aPL），导致血栓形成状态。aPL 诱导内皮细胞、单核细胞和血小板过度激活，同时伴有止血/凝血、纤维蛋白溶解系统和补体激活的异常。在有血栓形成和产科表现的 APS 患者中发现纤维蛋白溶解受损。纤溶酶原激活物抑制剂-1 和凝血酶激活的纤维蛋白溶解抑制剂水平升高，以及针对 annexin-2、组织型纤溶酶原激活物和纤溶酶的 aPL 的存在，导致这些患者的纤维蛋白溶解活性受损。此外，提出了纤维蛋白形态的不利改变，即纤维蛋白不易被纤维蛋白溶解，作为 APS 中的一种新的促血栓形成机制。本综述旨在总结目前关于 APS 患者纤维蛋白溶解受损相关机制的认识。我们还提供了一个临床实践中的病例，说明了 APS 患者纤维蛋白溶解受损的真实情况。
