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获得性血友病A

Acquired hemophilia A.

作者信息

Pandey Yadav, Atwal Dinesh, Konda Manojna, Roy Arya, Sasapu Appalanaidu

机构信息

Department of Internal Medicine, University of Arkansas for Medical SciencesLittle RockArkansas.

Department of Hematology and Oncology, University of Arkansas for Medical SciencesLittle RockArkansas.

出版信息

Proc (Bayl Univ Med Cent). 2019 Nov 20;33(1):71-74. doi: 10.1080/08998280.2019.1689024. eCollection 2020 Jan.

Abstract

Acquired inhibitors of coagulation are a group of rare but potentially life-threatening blood disorders characterized by the presence of autoantibodies directed against clotting factor. Autoantibody against factor VIII is the most common form of clotting factor inhibitor, a condition also known as acquired hemophilia A. We present a clinical series of nine patients diagnosed and treated for acquired hemophilia A at our institution. Among these nine patients, there were five men and four women with a median age of 64 years. All patients presented with bleeding diathesis. Factor eight inhibitor bypassing agent and/or recombinant factor VIIa were predominantly used for control of active bleeding. For elimination of autoantibodies, either steroids alone or the combination of steroids with rituximab or oral cyclophosphamide was used. Despite aggressive measures, two of the patients had a poor outcome; seven of the nine patients (77%) had a good clinical outcome. Acquired hemophilia A should be strongly suspected in any patient presenting with bleeding and a prolonged activated partial thromboplastin time. Early initiation of factor bypassing agents such as activated prothrombin complex concentrates or recombinant factor VIIa, along with the use of immunosuppressive agents, can be lifesaving.

摘要

获得性凝血抑制剂是一组罕见但可能危及生命的血液疾病,其特征是存在针对凝血因子的自身抗体。抗凝血因子VIII自身抗体是凝血因子抑制剂最常见的形式,这种情况也称为获得性血友病A。我们展示了在我们机构诊断和治疗的9例获得性血友病A患者的临床系列。这9例患者中,有5名男性和4名女性,中位年龄为64岁。所有患者均有出血倾向。凝血因子VIII抑制剂旁路制剂和/或重组凝血因子VIIa主要用于控制活动性出血。为了消除自身抗体,单独使用类固醇或类固醇与利妥昔单抗或口服环磷酰胺联合使用。尽管采取了积极措施,仍有2例患者预后不良;9例患者中有7例(77%)临床预后良好。对于任何出现出血且活化部分凝血活酶时间延长的患者,应高度怀疑获得性血友病A。早期启用凝血因子旁路制剂,如活化凝血酶原复合物浓缩物或重组凝血因子VIIa,同时使用免疫抑制剂,可挽救生命。

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Acquired hemophilia A.获得性血友病A
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Acquired haemophilia: an overview for clinical practice.获得性血友病:临床实践概述。
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Treatment of acquired haemophilia with recombinant activated FVII: a critical appraisal.
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