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HIV 相关和特发性获得性血友病 A:来自南非开普敦的单中心病例系列。

HIV-associated and idiopathic-acquired haemophilia A: A single-centre case series from Cape Town, South Africa.

机构信息

1Department of Medicine, Division of Clinical Haematology, University of Cape Town, Cape Town, South Africa.

出版信息

Neth J Med. 2020 Jul;78(4):196-201.

Abstract

Acquired haemophilia A is a rare coagulation disorder, which can lead to life-threatening haemorrhages if not identified and treated promptly. It is characterised by the presence of autoantibodies (inhibitors) to factor VIII. Acquired haemophilia A associated with HIV is a rare but well described phenomenon with limited directions to its management. We comparatively describe four patients - two with HIV and two without - that presented with unusual bleeding episodes with a prolonged activated partial thromboplastin time secondary to factor VIII inhibitors. An empiric observation is that the patients with acquired haemophilia A associated with HIV had higher antibody titres at presentation, that required more prolonged immunosuppressive therapy to induce remission.

摘要

获得性血友病 A 是一种罕见的凝血障碍疾病,如果不能及时识别和治疗,可能导致危及生命的出血。其特征是存在针对因子 VIII 的自身抗体(抑制剂)。与 HIV 相关的获得性血友病 A 是一种罕见但已有充分描述的现象,其治疗方法有限。我们比较描述了四位患者 - 两位 HIV 阳性,两位 HIV 阴性 - 他们均因因子 VIII 抑制剂而出现不寻常的出血发作和延长的激活部分凝血活酶时间。一个经验性观察是,与 HIV 相关的获得性血友病 A 患者在发病时抗体滴度更高,需要更长时间的免疫抑制治疗才能诱导缓解。

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