Zablonski Kevin G, Rajkumar Aarthi, Nayak Lalitha
University Hospitals, Seidman Cancer Center Cleveland Ohio USA.
Department of Internal Medicine Case Western Reserve University Cleveland Ohio USA.
EJHaem. 2024 Jun 29;5(4):833-837. doi: 10.1002/jha2.969. eCollection 2024 Aug.
Acquired von Willebrand syndrome (AVWS) is a bleeding disorder in which an underlying condition induces a quantitative or qualitative deficiency in the von Willebrand factor. This case demonstrates the rare diagnosis of AVWS due to an Immunoglobulin G monoclonal gammopathy in an elderly woman who presented with significant gastrointestinal bleeding. Originally thought to be type 1 von Willebrand disease, this case provides a cautious example to clinicians that without a detailed history or an understanding of the associated laboratory work-up, AVWS may be missed with potentially fatal consequences. Fortunately, AVWS was recognized and treated with intravenous immunoglobulin with a resolution of bleeding.
获得性血管性血友病综合征(AVWS)是一种出血性疾病,其中潜在疾病会导致血管性血友病因子出现数量或质量上的缺陷。本病例展示了一名老年女性因免疫球蛋白G单克隆丙种球蛋白病导致AVWS这一罕见诊断,该患者出现了严重的胃肠道出血。最初被认为是1型血管性血友病,此病例为临床医生提供了一个警示案例,即如果没有详细的病史或对相关实验室检查的了解,可能会漏诊AVWS,从而带来潜在的致命后果。幸运的是,AVWS得到了识别,并通过静脉注射免疫球蛋白进行治疗,出血症状得以缓解。