艾滋病毒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。

4区 医学 Q3 Medicine
Netherlands Journal of Medicine Pub Date : 2020-07-01
R Shein, J du Toit, M Goeijenbier, C du Toit, Estelle Verburgh
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引用次数: 0

摘要

获得性血友病A是一种罕见的凝血障碍,如果不及时发现和治疗,可导致危及生命的出血。其特点是存在对因子VIII的自身抗体(抑制剂)。与HIV相关的获得性血友病A是一种罕见但描述良好的现象,其管理方向有限。我们比较描述了四名患者-两名与艾滋病毒和两名没有-提出了不寻常的出血事件与延长活化部分凝血活素时间继发因子VIII抑制剂。一项经验性观察表明,与HIV相关的获得性血友病A患者在发病时抗体滴度较高,这需要更长的免疫抑制治疗来诱导缓解。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
HIV-associated and idiopathic-acquired haemophilia A: A single-centre case series from Cape Town, South Africa.

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.

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来源期刊
Netherlands Journal of Medicine
Netherlands Journal of Medicine 医学-医学:内科
自引率
0.00%
发文量
0
审稿时长
6-12 weeks
期刊介绍: The Netherlands Journal of Medicine publishes papers in all relevant fields of internal medicine. In addition to reports of original clinical and experimental studies, reviews on topics of interest or importance, case reports, book reviews and letters to the editor are welcomed.
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