Waisayarat Jariya, Plumworasawat Sirithep, Vilaiyuk Soamarat, Sirachainan Nongnuch
Department of Pathology, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
Department of Pediatrics, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
Vasc Health Risk Manag. 2019 Aug 7;15:253-258. doi: 10.2147/VHRM.S197638. eCollection 2019.
Antiphospholipid syndrome (APS) is an autoantibody-mediated acquired thrombophilia characterized by venous and/or arterial thromboses, pregnancy morbidity (predominantly repeated fetal losses), and the presence of phospholipid antibodies. The estimated annual incidence of APS is 5 new cases per 100,000 people. The most common thrombotic events in patients with APS in order of frequency are stroke, transient ischemic attack, deep vein thrombosis, and pulmonary embolism. Patients with APS may develop an intracardiac thrombus, which is a life-threatening complication with a high risk of increased morbidity and mortality; however, it is treatable by surgical removal, extensive anticoagulant administration, and prevention of other complications. Catastrophic APS, which is a rare and severe condition diagnosed based on rapidly progressive thromboembolic events involving three or more organs, systems, or tissues, occurs in less than 1% of all patients with APS. We herein report an autopsy case of catastrophic APS in a 12-year-old Thai boy with multiple thromboembolic events including intracardiac thrombus formation with a positive lupus anticoagulant test result. To the best of our knowledge, this is the youngest reported patient with APS to date.
抗磷脂综合征（APS）是一种自身抗体介导的获得性易栓症，其特征为静脉和/或动脉血栓形成、妊娠并发症（主要是反复流产）以及存在磷脂抗体。APS的估计年发病率为每10万人中有5例新发病例。APS患者中最常见的血栓形成事件按发生频率依次为中风、短暂性脑缺血发作、深静脉血栓形成和肺栓塞。APS患者可能会形成心内血栓，这是一种危及生命的并发症，具有较高的发病率和死亡率增加的风险；然而，可通过手术切除、大量使用抗凝剂以及预防其他并发症来进行治疗。灾难性抗磷脂综合征是一种罕见且严重的疾病，根据涉及三个或更多器官、系统或组织的快速进展性血栓栓塞事件进行诊断，在所有APS患者中发生率不到1%。我们在此报告一例12岁泰国男孩灾难性抗磷脂综合征的尸检病例，该男孩发生了多次血栓栓塞事件，包括心内血栓形成，狼疮抗凝物检测结果呈阳性。据我们所知，这是迄今为止报道的最年轻的APS患者。
