Qiao Jesse, Bailly Jenique, Opie Jessica
Department of Pathology and Laboratory Medicine, University of California, Irvine, USA.
Division of Haematology, Department of Pathology, University of Cape Town and National Health Laboratory Service, Groote Schuur Hospital, Cape Town, South Africa.
Clin Appl Thromb Hemost. 2024 Jan-Dec;30:10760296241306751. doi: 10.1177/10760296241306751.
Antiphospholipid syndrome (APS) is an autoimmune disorder characterized by antiphospholipid antibodies associated with thrombosis and pregnancy complications. Catastrophic APS is a severe form involving multiple organ systems with a high mortality rate. The pathogenesis involves antiphospholipid antibodies which target phospholipid-binding proteins and damage endothelial cells thus activating coagulation, triggering a pro-thrombotic state. Laboratory tests for antiphospholipid antibody detection include lupus anticoagulant testing in the coagulation laboratory and serological detection of anticardiolipin and anti-beta 2 glycoprotein I antibodies. Despite recent updates in the diagnostic criteria for APS the recent decades and our improved knowledge of this disease, there remain several key issues pertaining to diagnosis and testing with potential implications to patient management. Here we briefly review APS pathophysiology, strengths and weaknesses of classification criteria, laboratory challenges leading to test interpretation, and clinical management of this complex condition.
抗磷脂综合征(APS)是一种自身免疫性疾病,其特征是抗磷脂抗体与血栓形成和妊娠并发症相关。灾难性抗磷脂综合征是一种严重形式,累及多个器官系统,死亡率很高。发病机制涉及抗磷脂抗体,这些抗体靶向磷脂结合蛋白并损伤内皮细胞,从而激活凝血,引发血栓前状态。抗磷脂抗体检测的实验室检查包括凝血实验室的狼疮抗凝物检测以及抗心磷脂和抗β2糖蛋白I抗体的血清学检测。尽管近几十年来抗磷脂综合征的诊断标准有了更新,且我们对该疾病的认识有所提高,但在诊断和检测方面仍存在几个关键问题,可能对患者管理产生影响。在此,我们简要回顾抗磷脂综合征的病理生理学、分类标准的优缺点、导致检测结果解读困难的实验室挑战以及这种复杂疾病的临床管理。