Department of Clinical and Experimental Medicine, Federico II University Hospital, Naples, Italy.
Semin Thromb Hemost. 2012 Jul;38(5):433-46. doi: 10.1055/s-0032-1315757. Epub 2012 Jun 27.
Acquired hemophilia A (AHA) is a rare, but often severe, bleeding disorder caused by autoantibodies against clotting factor VIII (FVIII). AHA occurs more frequently in the elderly and in association with several conditions, such as malignancies, autoimmune diseases, postpartum, or drug exposure; however, about half of the cases remain idiopathic. At variance with congenital hemophilia, where hemarthroses are the most common bleeding symptoms, hemorrhages in AHA involving soft tissues (muscle, skin) are more frequently reported. AHA is diagnosed in patients: with negative personal or family bleeding history; in which prolonged activated partial thromboplastin time is not corrected after mixing and incubating equal volumes of patient and normal plasma for ~2 hours at 37°C; FVIII levels are reduced; and a specific FVIII-inhibiting activity is detected. Prompt recognition and treatment of AHA are mandatory, as inadequate management and complications of the disease are associated with high mortality rates. Therapeutic approaches should aim to control acute bleeds, eradicate FVIII-autoantibody production, treat associated diseases, and when possible, eliminate them. Present knowledge about this often overlooked and challenging condition has significantly increased following establishment of recent national and international studies, as will also be reviewed in this article.
获得性血友病 A（AHA）是一种罕见但常严重的出血性疾病，由针对凝血因子 VIII（FVIII）的自身抗体引起。AHA 在老年人中更常见，与多种情况相关，如恶性肿瘤、自身免疫性疾病、产后或药物暴露；然而，约一半的病例仍为特发性。与先天性血友病不同，关节积血是最常见的出血症状，AHA 中涉及软组织（肌肉、皮肤）的出血更为常见。AHA 诊断基于以下患者特征：无个人或家族出血史；混合患者和正常血浆等量并在 37°C 孵育约 2 小时后，延长的激活部分凝血活酶时间未得到纠正；FVIII 水平降低；并检测到特定的 FVIII 抑制活性。必须及时识别和治疗 AHA，因为疾病管理不当和并发症与高死亡率相关。治疗方法应旨在控制急性出血、消除 FVIII-自身抗体产生、治疗相关疾病，并在可能时消除这些疾病。随着最近的国家和国际研究的开展，对这种常被忽视且具有挑战性的疾病的认识显著提高，本文也将对此进行综述。
