Halbertsma F J, van der Linden P W, Mauser-Bunschoten E P
Kennemer Gasthuis, Department of Internal Medicine, Haarlem, Netherlands.
Neth J Med. 1998 May;52(5):190-2. doi: 10.1016/s0300-2977(98)00024-2.
Haemophilia A is usually a genetic deficiency of coagulation factor VIII (F VIII). The development of antibodies against F VIII is a well known and frequent complication in the treatment of haemophilia A. Rarely, a F VIII inhibitor arises spontaneously, causing a condition which is known as acquired haemophilia A. We describe a patient with acquired haemophilia A and pemphigus, who presented with spontaneous haematomas of the extremities. Laboratory tests showed an activated partial thromboplastin time (aPTT) of 71 s (normal: 26-36 s), a F VIII concentration of 9% (normal: 60-140%), and a F VIII inhibitor-activity of 7.5 Bethesda Units/ml (B.U./ml, normal: 0). The haematomas disappeared within a few days and the laboratory tests normalized within 6 weeks, after administration of a booster of oral corticosteroids. One and a half years after the corticosteroids were stopped, both the clinical and the laboratory course of the patient has been uneventful. As far as we know, the combination of acquired haemophilia A and pemphigus has been reported in the literature only three times before. The diagnosis acquired haemophilia A should be considered in a patient presenting with a newly arisen haemorrhagic diathesis.
甲型血友病通常是凝血因子 VIII（F VIII）的遗传性缺乏症。在甲型血友病的治疗中，针对 F VIII 的抗体产生是一种众所周知且常见的并发症。罕见的是，F VIII 抑制剂会自发出现，导致一种被称为获得性甲型血友病的病症。我们描述了一名患有获得性甲型血友病和天疱疮的患者，该患者出现了四肢自发性血肿。实验室检查显示活化部分凝血活酶时间（aPTT）为 71 秒（正常：26 - 36 秒），F VIII 浓度为 9%（正常：60 - 140%），F VIII 抑制剂活性为 7.5 贝塞斯达单位/毫升（B.U./ml，正常：0）。口服皮质类固醇加强剂量给药后，血肿在数天内消失，实验室检查在 6 周内恢复正常。停用皮质类固醇一年半后，患者的临床和实验室检查过程均无异常。据我们所知，获得性甲型血友病和天疱疮的组合此前在文献中仅被报道过三次。对于出现新发性出血素质的患者，应考虑诊断为获得性甲型血友病。
