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单用皮质类固醇根除一名老年特发性获得性甲型血友病女性患者体内Ⅷ因子抑制物的疗效:病例描述

Efficacy of Corticosteroids Alone in the Eradication of Factor VIII Inhibitor in an Old Female with Idiopathic Acquired Haemophilia A: Description of a Case.

作者信息

Girelli Francesco, Biasoli Chiara, Bassi Bruna, Bagioni Franco, Bondi Gabriele, Camporesi Claudio, Gardelli Lucia, Mazzeo Vincenzo, Nizzoli Maurizio

机构信息

Specialistic Medicine Department, Internal Medicine Unit, Rheumatology Service, GB Morgagni Hospital, 47121 Forlì, Italy.

出版信息

Case Rep Rheumatol. 2012;2012:310730. doi: 10.1155/2012/310730. Epub 2012 Aug 29.

Abstract

Acquired haemophilia A (AHA) is a rare and serious disorder mainly affecting elderly patients. It is caused by the production of autoantibodies directed against coagulation factors; patients present with spontaneous bleeding, potentially fatal, in the absence of familial or personal history. Autoimmune disorders, infections, solid and hematologic tumors, and drugs are predisposing factors, but up to 50 percent of cases remain unexplained. The diagnosis of AHA is confirmed by specific laboratory tests; and the therapy is a clinical challenge, due to the fact that older patients are often affected by comorbidities. By passing agents may be used when persistent bleeding or haemodynamic instability is observed; corticosteroids, alone or with immunosuppressive therapy, are necessary to inhibit the production of the autoantibodies. We describe a case in which steroids in monotherapy successfully, safely, and persistently inhibited the production of anti-Factor VIII antibodies, in an old patient admitted after rheumatologic consult.

摘要

获得性血友病A(AHA)是一种罕见且严重的疾病,主要影响老年患者。它是由针对凝血因子的自身抗体产生所引起的;患者在没有家族或个人病史的情况下出现自发性出血,可能会危及生命。自身免疫性疾病、感染、实体和血液系统肿瘤以及药物都是诱发因素,但高达50%的病例仍原因不明。AHA的诊断通过特定实验室检查得以证实;由于老年患者常伴有合并症,因此治疗是一项临床挑战。当观察到持续性出血或血流动力学不稳定时可使用旁路制剂;单独使用皮质类固醇或与免疫抑制治疗联合使用,对于抑制自身抗体的产生是必要的。我们描述了一例在风湿科会诊后收治的老年患者,其中单药使用类固醇成功、安全且持久地抑制了抗凝血因子VIII抗体的产生。

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本文引用的文献

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