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A case of acquired hemophilia A with massive hemothorax.

机译:一例获得性血友病A伴大量血胸。

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Acquired hemophilia A (AHA) is an uncommon but potentially life-threatening hemorrhagic disorder caused by the development of an inhibitor against coagulation factor VIII (FVIII). AHA is very rare, affecting approximately 1 in 1?million individuals. However, the incidence may actually be higher, because diagnosis is difficult and the disease can be overlooked. We report a case of an 80-year-old man who presented with sudden onset of severe hemothorax. The patient was diagnosed with presumed AHA based on acute onset of bleeding symptoms and unexplained isolated prolonged activated partial thromboplastin time. Diagnosis was definitely established by demonstrating a decrease in FVIII activity, presence of FVIII inhibitor activity, and normal von Willebrand factor. The patient was successfully treated with recombinant activated coagulation factor VII and transcatheter artery embolization of the intercostal arteries.
机译:获得性血友病A(AHA)是一种罕见的但可能威胁生命的出血性疾病,由凝血因子VIII(FVIII)抑制剂的开发引起。 AHA非常罕见,大约每百万人口中就有1个人受到影响。但是,由于诊断困难并且可以忽略该疾病,因此发病率实际上可能更高。我们报告了一例80岁男子,他突然出现严重的血胸。根据出血症状的急性发作和无法解释的孤立的延长的活化部分凝血活酶时间的延长,诊断为该患者为AHA。通过证实FVIII活性降低,FVIII抑制剂活性存在以及正常的von Willebrand因子,可以明确诊断。该患者已成功用重组活化凝血因子VII和肋间动脉的经导管动脉栓塞治疗。

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