rFⅦa联合强化免疫抑制治疗获得性血友病A的回顾性临床研究  

A Retrospective Clinical Study of rFⅦa combined with Immunosuppressive Therapy in the Treatment of Acquired Hemophilia A

作　　者：周莉[1] 张馨慧 冯尚龙 胡星[1] 赵娜 郑昌成 吴竞生 ZHOU Li;ZHANG Xin-hui;FENG Shang-long;HU Xing;ZHAO Na;ZHENG Chang-chegn;WU Jing-sheng(The First Affiliated Hospital of USTC,Division of Life Sciences and Medicine,University of Science and Technology of China,Hefei,230001,China;Provincial Hospital Affiliated to Anhui Medical University,Hefei,230032,China)

机构地区：[1]中国科学技术大学附属第一医院(安徽省立医院),合肥230001 [2]安徽医科大学附属省立医院,合肥230032

出　　处：《血栓与止血学》2021年第2期181-185,共5页Chinese Journal of Thrombosis and Hemostasis

基　　金：安徽省重点研究与开发计划项目(201904a07020094);安徽省自然科学基金面上项目(2008085MH249);中央高校基金科大新医学项目(No.WK9110000138)。

摘　　要：目的总结获得性血友病A的临床表现、病因、治疗方案及预后。治疗包括rFⅦa联合强化免疫抑制治疗(IST)。方法回顾性分析2017年6月1日~2020年5月30日在中国科学技术大学附属第一医院确诊AHA病例12例,总结其临床表现、病因、治疗方案及预后。治疗包括止血替代治疗和环磷酰胺(CTX)联合糖皮质激素为基础的强化免疫抑制治疗(IST)。按期复查FⅦI活性及FⅦI抑制物滴度,免疫抑制剂逐渐减量至小剂量维持直至停药并进行疗效评价。结果12例患者其中10例分别在1-9个月达到了CR或PR状态,仅2例患者采用利妥昔单抗治疗。2例患者未坚持复查,10例患者后期随访至少1个月以上,9例(75.0%)患者处于持续CR状态。1例患者缓解后复发,经治疗再缓解。至总结时,9例CR患者抗体滴度转阴,中位转阴时间为3个月(1-18个月)。无患者发生骨髓抑制等不良反应。结论采用包括人重组Ⅶ因子的止血治疗方案及CTX联合糖皮质激素为基础的IST方案可有效止血并在短期内去除抗体达到有效治疗,且无骨髓抑制等不良反应。Objective Summarize the clinical manifestations,causes,treatment plans and prognosis of obtained haemophilia A.Treatment includes rFⅦa combined enhanced immunosuppressive therapy(IST).Methods Retrospective analysis of 12 confirmed AHA cases at the First Hospital affiliated with China University of Science and Technology from June 1,2017 to May 30,2020,summarized their clinical manifestations,ethiopaths,treatment options and prognosis.Treatments include hemostase replacement therapy and cyclophosphamide(CTX)combined with glucoticoid-based intensive immunosuppressive therapy(IST).The FⅦI activity and FⅦI inhibitor titration were reviewed on a timely date,and the immunosuppressant was gradually reduced to a small dose to maintain until the drug was stopped and the efficacy evaluation was carried out.Results Of the 12 patients,10 reached CR or PR in 1-9 months,and only 2 patients were treated with lyxid monoantigen.2 patients did not insist on review,10 patients followed up at least 1 month later,9 patients(75.0%)were in a continuous CR state.1 patient relapsed after remission,after treatment and remission.At the end of the day,9 cr patients had antibody titration,with a moderate transsethropping time of 3 months(1-18 months).No adverse reactions such as bone marrow inhibition occurred in patients.Conclusion Use of hemostrogenic treatment program including human recombinantⅦfactor and CTX combined glucoticoid-based IST program can effectively stop bleeding and remove antibodies in a short period of time to achieve effective treatment,and no adverse reactions such as bone marrow inhibition.

关 键 词：获得性血友病A 止血治疗 强化免疫抑制治疗 

分 类 号：R554[医药卫生—血液循环系统疾病]