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静脉注射免疫球蛋白可使单克隆丙种球蛋白病所致获得性血管性血友病综合征暂时改善:一例报告

Intravenous Immunoglobulin offers temporary improvement in acquired von Willebrand syndrome due to monoclonal gammopathy: A case report.

作者信息

Zablonski Kevin G, Rajkumar Aarthi, Nayak Lalitha

机构信息

University Hospitals, Seidman Cancer Center Cleveland Ohio USA.

Department of Internal Medicine Case Western Reserve University Cleveland Ohio USA.

出版信息

EJHaem. 2024 Jun 29;5(4):833-837. doi: 10.1002/jha2.969. eCollection 2024 Aug.

Abstract

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.

摘要

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。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/702f/11327770/83d989275d2a/JHA2-5-833-g001.jpg

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