Kevin G. Zablonski, Aarthi Rajkumar, Lalitha Nayak
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引用次数: 0
摘要
获得性冯-维勒布兰德综合征(AVWS)是一种出血性疾病,由潜在疾病引起冯-维勒布兰德因子定量或定性缺乏。本病例显示,在一名出现严重消化道出血的老年妇女身上,因免疫球蛋白 G 单克隆性腺病而确诊为 AVWS 的病例十分罕见。该病例最初被认为是 1 型冯-维勒布兰德病,但它为临床医生提供了一个谨慎的范例,即如果没有详细的病史或对相关实验室检查的了解,AVWS 可能会被漏诊,并可能造成致命后果。幸运的是,AVWS 被识别出来并接受了静脉注射免疫球蛋白治疗,出血症状得到缓解。
Intravenous Immunoglobulin offers temporary improvement in acquired von Willebrand syndrome due to monoclonal gammopathy: A case report
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.