获得性血友病A的免疫抑制治疗研究进展  

Advance in immunosuppressive therapy for acquired hemophilia A

作　　者：傅玉晗(综述) 陈姝(审校)[1] FU Yuhan;CHEN Shu(Department of Hematological Internal Medicine,Second Affiliated Hospital,Chongqing Medical University,Chongqing 400010,China)

机构地区：[1]重庆医科大学附属第二医院血液内科,重庆400010

出　　处：《现代医药卫生》2023年第10期1757-1761,共5页Journal of Modern Medicine & Health

摘　　要：获得性血友病A(AHA)是由于循环中产生针对凝血因子Ⅷ的自身抗体导致凝血因子Ⅷ活性下降的罕见性出血性疾病。近年来随着对该病文献报道的增加及国内外几个大型登记研究的临床数据报告,人们对该病的认识逐渐提高,但由于疾病本身相对罕见,仍存在认识不足、诊治延迟的情况,其中治疗主要包括三方面:控制急性期出血、清除抑制物、治疗原发疾病或去除诱因,清除抑制物的免疫抑制治疗(IST)被认为是治疗的基石。近年有研究显示IST相关死亡率甚至超过直接由出血导致的死亡率,AHA的IST仍极具挑战性,需要不断总结经验并探索优化的治疗方案。该文将主要从AHA基本临床特征、抑制物特点、目前主要的IST方案及最新的治疗方案进行综述。Acquired hemophilia A(AHA)is a rare bleeding disorder caused by the circulating autoantibodies directedly against coagulation factorⅧ,resulting in decreased activity of coagulation factorⅧ.In recent years,awareness of the disease has gradually increased due to the increased reporting of the disease and data reported from several large registry studies in China and abroad,but due to the relative rarity of the disease itself,there is still a lack of awareness and delay in diagnosis and treatment.Its treatment consists of three main aspects:control of bleeding in the acute phase,eradication of inhibitors,and treatment of the primary disease or removal of the causative agent.Immunosuppressive treatment(IST)to eradicate inhibitors is the therapeutic foundation.However,recent studies have shown that IST-related mortality exceeds even that caused directly by bleeding,so the IST of AHA is still very challenging and requires continuous experience and exploration of optimal treatment protocols.In this paper,we will focus on the basic clinical features,characteristics of coagulation factorⅧinhibitors in acquired hemophilia A,the current major immunosuppressive treatment options,and the latest treatment options.

关 键 词：获得性血友病A 免疫抑制疗法 凝血因子Ⅷ 出血性疾病 综述 

分 类 号：R55[医药卫生—血液循环系统疾病]