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获得性血友病合并大疱性类天疱疮:一例报告

Acquired hemophilia associated with bullous pemphigoid: a case report.

作者信息

Qiu Xiangning, Zhang Guiying, Xiao Rong, Zhang Jing, Zhou Ying, Li Ganqun, Yan Kailin, Lu Qianjin

机构信息

Department of Dermatology, Second Xiangya Hospital Central South University, No. 139, Renmin Road, Changsha, Hunan 410011, PR China.

出版信息

Int J Clin Exp Pathol. 2012;5(1):102-4. Epub 2012 Jan 5.

Abstract

The development of factor VIII inhibitors in non-hemophilic patients is rare and may occur in healthy individuals, mostly elderly and women in postpartum period, and in patients with malignant neoplasia or autoimmune diseases, such as bullous pemphigoid. We described the case of a 60-year-old female patient who developed bullous pemphigoid for 3 month and presented with bleeding tendency and hematoma in the tongue. Therapy with methylprednisolone, cyclophosphamide, intravenous immunoglobulin and factor VIII reposition was instituted, resulting in a remission of the bleeding and negativity for antibodies against factor VIII titers. We concluded that, despite its rarity, the presence of acquired factor VIII inhibitors should be investigated when patients with autoimmune diseases develop bleeding manifestations.

摘要

非血友病患者中因子VIII抑制剂的发生较为罕见,可能出现在健康个体中,主要是老年人和产后女性,以及患有恶性肿瘤或自身免疫性疾病(如大疱性类天疱疮)的患者。我们描述了一名60岁女性患者的病例,该患者患大疱性类天疱疮3个月,出现出血倾向和舌部血肿。采用甲泼尼龙、环磷酰胺、静脉注射免疫球蛋白和补充因子VIII进行治疗,出血症状缓解,因子VIII抗体滴度转阴。我们得出结论,尽管罕见,但自身免疫性疾病患者出现出血表现时,应调查是否存在获得性因子VIII抑制剂。

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