单倍体造血干细胞移植治疗儿童急性淋巴细胞白血病后并发获得性血友病1例并文献复习  

Acquired hemophilia following haploidentical hematopoietic stem cell transplantation for acute lymphoblastic leukemia in children: one case report and literature review

作　　者：景沼贺 王英洁 买钰淼 孙盼 董芃芃 刘健 JING Zhaohe;WANG Yingjie;MAI Yumiao;SUN Pan;DONG Pengpeng;LIU Jian(Department of Pediatrics,the First Affiliated Hospital of Zhengzhou University,Zhengzhou,Henan 450052,China)

机构地区：[1]郑州大学第一附属医院儿科,河南郑州450052

出　　处：《中华全科医学》2024年第5期900-902,共3页Chinese Journal of General Practice

基　　金：郑州大学青年教师基础研究培育基金项目(JC21854036)。

摘　　要：获得性血友病(AH)是由抗凝血因子的自身抗体引起的一种罕见疾病。本文报道1例急性淋巴细胞白血病(T系,高危)患儿,经单倍体造血干细胞移植(父供女,HLA 5/10)治疗后并发获得性血友病,同时结合文献进行复习。该患儿出现凝血因子Ⅸ、Ⅺ和Ⅻ活性降低,及Ⅸ因子抑制物滴度升高,临床较少见,经及时诊断并给予甲泼尼龙联合环孢素免疫抑制治疗后,患儿症状好转出院并继续规律服用甲泼尼龙及环孢素。患儿出院4个月后症状再发,予甲泼尼龙加量联合环孢素免疫抑制治疗后,患儿病情好转。随访至2023年3月患儿症状无再发。Acquired hemophilia(AH)is a rare disease caused by autoantibodies against factors.We reported a case of acute lymphoblastic leukemia(T-lineage,high-risk)in a child who developed acquired hemophilia after haploidentical hematopoietic stem cell transplantation(paternal donor,HLA 5/10)and reviewed the relevant literature.Laboratory tests showed decreased coagulation factorⅨactivity(FⅨ:C),FⅪ:C,and FⅫ:C,and an elevated FⅨinhibitor titer,which is rare the clinic.After diagnosis,the child was treated with methylprednisolone in combination with cyclosporine A.With improvement of symptoms,the patient was discharged and continued to take cyclosporine A and methylprednisolone regularly.Four months after discharge,symptoms recurred,and the treatment regimen was changed to double-dose methylprednisolone combined with cyclosporine A immunosuppressive therapy,and the child was discharged with improvement.There was no recurrence of symptoms until March 2023.

关 键 词：获得性血友病 单倍体造血干细胞移植 儿童 凝血因子Ⅸ抗体 

分 类 号：R733.7[医药卫生—肿瘤] R554.1[医药卫生—临床医学]