Diagnosis and treatment of acquired haemophilia
Diagnosis and treatment of acquired haemophilia
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获得性血友病的诊断和治疗
DOI：
10.1111/j.1365-2516.2010.02232.x
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发表时间：
2010
期刊：
影响因子：
3.9
通讯作者：
F. de Cataldo
中科院分区：
文献类型：
--
作者：
F. Baudo;T. Caimi;F. de Cataldo
摘要：获得性血友病（AH）是一种自身免疫综合征，其特征是家族史和个人史阴性的患者发生急性出血，并伴有因子VIII耗竭。 发病率为1.6 × 106人/年。 AH与自身免疫性疾病、实体瘤、淋巴增生性疾病、妊娠相关; 50%的病例为特发性。自发性或轻微创伤后严重出血伴活化部分凝血活酶时间延长（未通过与正常血浆孵育校正）和凝血酶原时间正常是诊断标志。治疗的目标是控制出血和抑制抑制剂。一线止血治疗包括重组凝血因子VIIa和活化凝血酶原复合物浓缩物。泼尼松±环磷酰胺和其他免疫抑制剂是抑制剂根除的标准干预。
Summary. Acquired haemophilia (AH) is an autoimmune syndrome characterized by acute bleeding in patients with negative family and personal history, and factor VIII depletion. Its incidence is 1.6 × 106 population per year. AH is associated with autoimmune diseases, solid tumours, lymphoprolipherative diseases, pregnancy; 50% of the cases idiopathic. Spontaneous or after minor trauma severe bleeding associated with a prolonged activated partial thromboplastin time, not corrected by incubation with normal plasma, with a normal prothrombin time are the diagnostic hallmarks. The goals of management are the control of bleeding and the suppression of inhibitor. First‐line haemostatic treatment includes recombinant factor VIIa and activated prothrombin complex concentrate. Prednisone ± cyclophosphamide and other immunosuppressive agents are the standard intervention for inhibitor eradication.