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获得性血友病A:三例病例及文献综述

Acquired Hemophilia A: Three Cases and Review of the Literature.

作者信息

Sakalli H El Bayed, Matrane W, Hamzaoui Z El, Oukkache B

出版信息

Clin Lab. 2019 Sep 1;65(9). doi: 10.7754/Clin.Lab.2019.190140.

DOI:10.7754/Clin.Lab.2019.190140
PMID:31532083
Abstract

BACKGROUND

Acquired hemophilia A (AHA) is an autoimmune disease caused by autoantibodies against coagulation factor VIII. It is a rare and potentially fatal and often underestimated pathology, mainly in the elderly person and for whom the rapidity of the diagnosis and the initiation of the background treatment are necessary. We reported three cases diagnosed in our hospital.

METHODS AND RESULTS

First case: A 55-year-old man, without personal or familial hemorrhagic case history. Admitted to the hospital with anemic and hemorrhagic cutaneous syndromes. His treatment included Transfusion support, concentrate F VIIIa, and corticosteroids with good clinical evolution. Second case: An 82-year-old man, without case history, admitted with cutaneous mucosal hemorrhagic Syndrome with hemorrhage of the puncture sites. Good evolution with treatment based on NovoSeven, corticosteroid, and cyclophosphamide in addition to transfusion support. Third case: A 52-year-old man, was followed for 3 years for pemphigoid. He was hospitalized for surgical Treatment of an extensive and painful hematoma of the anterior aspect of the right leg following a fall and treated with corticosteroid and NovoSeven.

CONCLUSIONS

Although rare, AHA must be diagnosed early, and may, at any time, commit to the vital prognosis by the appearance of serious hemorrhagic complications.

摘要

背景

获得性血友病A(AHA)是一种由针对凝血因子VIII的自身抗体引起的自身免疫性疾病。它是一种罕见且可能致命的疾病,常常被低估,主要发生在老年人中,对于他们而言,快速诊断和启动基础治疗是必要的。我们报告了在我院诊断的三例病例。

方法与结果

第一例:一名55岁男性,无个人或家族出血病史。因贫血和皮肤出血综合征入院。其治疗包括输血支持、F VIIIa浓缩剂和皮质类固醇,临床进展良好。第二例:一名82岁男性,无病史,因皮肤黏膜出血综合征伴穿刺部位出血入院。除输血支持外,基于诺其、皮质类固醇和环磷酰胺的治疗效果良好。第三例:一名52岁男性,患类天疱疮3年。因跌倒后右小腿前部出现广泛且疼痛的血肿而住院接受手术治疗,并用皮质类固醇和诺其进行治疗。

结论

尽管AHA罕见,但必须早期诊断,并且可能在任何时候因严重出血并发症的出现而危及生命预后。

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Acquired Hemophilia A: Three Cases and Review of the Literature.获得性血友病A:三例病例及文献综述
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[Bullous pemphigoid and acquired hemophilia].[大疱性类天疱疮与获得性血友病]
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Acquired Hemophilia of Unknown Etiology in an Elderly Man: Case Report.一名老年男性的不明病因获得性血友病:病例报告
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The first case of postpartum acquired hemophilia A in Korea.韩国首例产后获得性血友病 A 病例。
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[Preliminary experience with achieving immune tolerance in patients with hemophilia A and circulating factor VIII inhibitor].
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Acquired Factor VIII autoantibody: four cases demonstrating the heterogenous nature of this condition and problems involved in diagnosis and treatment.获得性因子VIII自身抗体:4例病例展示了这种疾病的异质性以及诊断和治疗中涉及的问题。
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