Tun Nay T, Krishnamurthy Mahesh, Snyder Richard
aDepartment of Internal Medicine bDivision of Nephrology, Department of Internal Medicine, Easton Hospital, Drexel University, School of Medicine, Easton, Pennsylvania, USA.
Blood Coagul Fibrinolysis. 2015 Mar;26(2):214-9. doi: 10.1097/MBC.0000000000000210.
Antiphospholipid syndrome (APS) and heparin-induced thrombocytopenia (HIT) are thrombotic disorders due to specific autoimmune-mediated antibodies. Catastrophic APS (CAPS), also known as Asherman's syndrome, is a life-threatening severe form of APS. Diagnostic criteria for CAPS include the development of a thrombotic event of three or more organs in less than a week with the presence of antiphospholipid antibodies and microvascular thrombosis on histology. Thrombocytopenia is seen in more than 60% of cases of CAPS. HIT is a life-threatening disorder with the clinical presentation of thrombocytopenia and arterial or venous thrombosis in patients who develop antibodies to heparin and platelet factor 4 typically within 10 days after starting heparin treatment. Due to the multiple similarities in clinical features and pathophysiology of CAPS and HIT, it has been postulated that these two antibody-mediated disorders may be related. We report two cases in which patients diagnosed with CAPS developed HIT very soon during the same admission as well as a case of a patient initially diagnosed with HIT who presented with CAPS years later.
抗磷脂综合征(APS)和肝素诱导的血小板减少症(HIT)是由特定自身免疫介导抗体引起的血栓形成性疾病。灾难性抗磷脂综合征(CAPS),也称为阿谢曼综合征,是一种危及生命的严重APS形式。CAPS的诊断标准包括在不到一周的时间内三个或更多器官发生血栓形成事件,同时存在抗磷脂抗体以及组织学上的微血管血栓形成。超过60%的CAPS病例可见血小板减少。HIT是一种危及生命的疾病,临床表现为血小板减少以及在开始肝素治疗后通常10天内产生肝素和血小板因子4抗体的患者出现动脉或静脉血栓形成。由于CAPS和HIT在临床特征和病理生理学方面存在多种相似之处,有人推测这两种抗体介导的疾病可能相关。我们报告了两例被诊断为CAPS的患者在同一住院期间很快发生HIT的病例,以及一例最初被诊断为HIT的患者数年后出现CAPS的病例。