Abstract:

Objective To summary and discuss the successful diagnosis and therapy to antibody mediatedrejection（AMR）post pediatric ABO-incompatible liver transplantation. Methods Abnormal graft function was found within 1 month after living donor liver transplantation. Then，liver biopsy were performed twice and human lymphocyte antigen（HLA）antibodies were detected. Results Acute rejection was revealed in both liver biopsies，intravenous methylprednisolone was used for anti-rejection therapy. AMR was diagnosed by positivity of C4d and strong expression of donor specific antibody（DSA）subsequently. The effective therapies were performedincluding combination of plasmapheresis and intravenous immunoglobulin（IVIG）on alternate days for 1 week，followed by single rituximab. Meanwhile，mycophenolate mofetil was used at the beginning. The graft function recovered to normal with decreasing of DSA. The patient has been followed for more than 2 years with well graftfunction. Conclusion AMR could be induced by ABO incompatible pediatric liver transplantation with serious consequence. AMR can be cured by conservative therapies and normal graft function could be achieved.

Key words: Pediatrics , ABO-incompatible , Living donor liver transplantation , Donor specific antibody, Antibody mediated rejection

摘要：

目的 探讨救治 1 例 ABO 血型不合儿童肝移植术后出现抗体介导的排斥反应（antibody mediated rejection，AMR）的成功经验。方法 ABO 血型不合儿童肝移植 1 例，患者行亲体肝移植术后 1 个月内出现肝功能异常，先后行 2 次肝穿活检，并检测人类白细胞抗原（human lymphocyte antigen，HLA）抗体。结果 肝穿结果均显示急性排斥反应，先后 2 次静脉甲泼尼龙冲击治疗无效。后依据 C4d 阳性、 HLA- Ⅱ类抗体强阳性，确诊为供体特异性抗体（donor specific antibody，DSA）导致的 AMR。在接受血浆置换与静脉用人免疫球蛋白（intravenous immunolobulin，IVIG）交替治疗 1 周后，应用利妥昔单抗治疗 1 次，在治疗初期加用吗替麦考酚酸酯治疗。治疗 4 周后，DSA 强度逐步下降，肝功能恢复至正常。随访 2 年余，肝功能正常。结论 ABO 血型不合的儿童肝移植可引起术后发生 AMR，后果严重，在明确诊断后，保守治疗可治愈 AMR，恢复移植肝功能正常。

关键词: 儿童 , ABO 血型不合 , 亲体肝移植 , 供体特异性抗体 , 抗体介导的排斥反应