获得性血友病A20例临床分析

A Clinical Analysis of 20 cases with Acquired Hemophilia A

目的探讨获得性血友病A(AHA)的临床特点与治疗方法,以期早期诊断和提高治疗效果。方法回顾性分析20例AHA的临床特点、实验室检查及治疗与转归。结果 20例AHA中位年龄65岁,以皮肤瘀斑和肌肉血肿为主要表现,多数患者无明确病因或诱因,3例接受单用皮质激素治疗,15例接受激素联合免疫抑制剂治疗,2例在免疫抑制剂基础上联合小剂量利妥昔单抗治疗,所有患者的出血倾向明显好转。结论 AHA以老年人多见,单纯的aPTT延长不能被1∶1的血浆纠正时应该考虑本病。治疗上以联合免疫制剂为主。对于难治性AHA,小剂量利妥昔单抗是一个很好的选择。

Objective Aim to early diagnosis and improving treatment effect through investigating the clinical features and therapeutic methods of acquired hemophilia A （AHA） ; Methods Retrospective analysis of the clinical course, laboratory result, treatment and outcome of 20 AHA ; Results The predominating manifestations of AHA are dermal ecchymosis and intramuscular hematoma with a median age of 65. Most patients have no underlying diseases. 3 and 15 patients received only cortieosteroids and combined cortieoste- roids and immunosuppressor, respectively. 2 patients received low dose rituximab plus combined corticosteroids and immunosuppressor. All patients have a good response in hemorrhagic tendency. Conclusions AHA is mainly seen in old patients, a diagnosis of AHA should be considered when isolated aPTI＇ prolongs which can not be corrected by equal normal plasma. Combined eorticosteroids and immunosuppressor is the first-line therapy for AHA, but low dose rituximab is a good option for refractory case.