【摘要】 目的 探讨血浆置换(plasma exchange, PE)治疗儿童亲属活体肝移植术后出现抗体介导排斥反应(antibody mediated rejection, AMR)的疗效。方法 以湖南省儿童医院1例因胆道闭锁、胆汁性肝硬化接受血型相合亲属活体肝移植，术后发生AMR的7月龄患儿为研究对象。该患儿肝移植术后一直使用他克莫司+泼尼松二联抗排斥治疗，术后1个月内出现以胆红素升高为主的肝功能异常，行移植肝穿刺活检，完善人类白细胞抗原(human lymphocyte antigen, HLA)抗体检测，并予血浆置换治疗。结果 肝穿刺活检结果提示急性排斥反应、补体C4d染色阳性，结合人类白细胞抗原(human lymphocyte antigen, HLA)Ⅱ类特异性抗体阳性这一结果，确诊为供体特异性抗体(donor specific antibody, DSA)导致的AMR。确诊后予甲泼尼龙静脉冲击+静脉丙种球蛋白+吗替麦考酚脂口服治疗，并调整相关免疫抑制药物剂量，效果不理想，后加用血浆置换治疗，每2日1次，共5次，治疗1个月后患儿肝功能逐步恢复正常。截至目前已随访3年，复查过程中行移植肝穿刺活检5次，显示排斥反应逐步减轻，肝功能基本维持正常，未出现AMR复发。结论 血型相容的儿童亲属活体肝移植也可发生术后AMR,确诊后尽早联合血浆置换辅助治疗可能更有利于肝移植术后急性AMR的恢复。更多还原

【Abstract】 Objective To explore the clinical efficacy of plasma exchange(PE) for antibody mediated rejection(AMR) after living relative donor liver transplantation. Methods Clinical data were retrospectively reviewed for a 7-month-old child with biliary atresia and biliary cirrhosis after living relative donor liver transplantation.Tacrolimus plus prednisone was prescribed for anti-rejection therapy after compatible blood type living relative donor liver transplantation.Liver function abnormality was caused predominantly by a postoperative elevation of bilirubin within 1 month.Then transplanted liver was biopsied and antibody test of human leukocyte antigen(HLA) performed. Results Liver biopsy revealed acute rejection reaction.Based upon the positive result of HLA II antibody and C4 d, it could be confirmed as donor specific antibody(DSA)-induced AMR.The regimen was composed of intravenous methylprednisolone pulse, immunoglobulin, mycophenolate and immunosuppressive agents.However, the effect was not satisfactory.And then PE was performed every 2 days for a total of 5 sessions.After 1-month treatment, liver function gradually normalized.Follow-up was conducted for 3 years.Transplanted liver was biopsied for 5 times and rejection gradually lessened and liver function basically stablized.There was no recrudescence of AMR. Conclusion Postoperative AMR occurs in children with compatible blood type living relative donor liver transplantation.Early treatment plus PE may be more beneficial to the recovery of acute AMR after liver transplantation.更多还原