Abstract:

Objective To analyze clinical characteristics of posttransplant lymphoproliferativedisorder （PTLD） after pediatric liver transplantation, and to summarize its clinical diagnosis and treatment experience. Methods We retrospectively analyzed the clinical characteristics, laboratory data, radiological data, pathological result, treatment, and prognosis of 18 pediatric PTLD patients after liver transplantation presenting toBeijing Friendship Hospital from January, 2017 to September, 2019. Results A total of 18 patients were included in this study. The median age at surgery was 15.9 months （range, 4.6 ~ 146.7）, and the median onset time of PTLD was 15.1 months （range, 4.2 ~ 30.1） postoperatively. 88.9% （16/18） of patients had superficial lymphadenopathy, 94.4% （17/18） had Epstein-Barr viremia, and 88.9% （16/18） was EBER positive. In 17 patients, positron emission computed tomography （PET）-CT revealed increased FDG metabolism in the associated enlarged lymph nodes. All 18 patients underwent immunosuppression reduction, and were treated with targeted therapy, chemotherapy, surgery and adoptiveimmunotherapy with EBV-CTLs （EBV-specific cytotoxic T-cells） according to the pathological type. One patient died and 17 had clinical remission. ConclusionThe increased incidence of PTLD after pediatric liver transplantation may be related to EBV infection and high level of immunosuppression. The possibility of PTLD should be consideredin patients with EB-Viremia and superficial lymphadenopathy but without nonspecific symptoms. Monitoring EBVDNA replication load and reducing the level of immunosuppression are important means to treat PTLD in children after liver transplantation. Early diagnosis and treatment are of great significance to the prognosis of PTLD. 

Key words:

Pediatric liver transplantation ,  , Posttransplant lymphoproliferative disorder ,  , Epstein-Barr Virus infection,  , Cell sorting,  , Reduction inimmunosuppression

摘要：

目的 总结儿童肝移植患者移植后淋巴组织增殖性疾病（posttransplant lymphoproliferative disorder，PTLD）的诊疗经验。方法 回顾性分析 2017 年 1 月至 2019 年 12 月期间就诊于首都医科大学附属北京友谊医院，行病理确诊 PTLD 的 18 例儿童肝移植患者的临床资料，收集并分析确诊 PTLD 的儿 童肝移植患者临床症状、实验室数据、影像学资料、病理结果、治疗方式和预后。结果 本研究纳入的18 例移植后 PTLD 患者中位移植年龄为 15.9（4.6 ~ 146.7）个月，手术后 PTLD 中位发病时间为 15.1（4.2 ~30.1）个月。88.9%（16/18）的 PTLD 患者存在浅表淋巴结肿大，94.4%（17/18）患者伴 EB 病毒血症，88.9% （16/18）患者病理学结果显示 EBER 原位杂交阳性。17 例患者正电子发射计算机断层显像（PET）-CT 提示相关肿大淋巴结 FDG 代谢增高。18 例患者均应用减停免疫抑制剂治疗，根据病理类型，PTLD 患者应用 靶向治疗、化疗、手术切除及 EBV-CTL 过继性免疫治疗。1 例患者死亡，17 例患者临床缓解。结论 儿童 肝移植术后 EBV 感染和免疫抑制水平较强可能导致 PTLD 的患病风险增加。非特异性症状合并 EB 病毒血症伴有浅表淋巴结肿大时，需考虑到 PTLD 的可能。监测 EBV DNA 复制载量和降低免疫抑制水平是治疗儿 童肝移植术后 PTLD 的重要手段，尽早诊断和治疗对 PTLD 患者的预后意义重大。

关键词:

儿童肝移植 , 移植后淋巴组织增殖性疾病 ,  , EBV 感染 , 细胞分选 , 免疫抑制减量