Objective: To summarize the clinical characteristics, prognostic factors and treatment outcomes of childhood aggressive mature B-cell lymphoma after liver transplantation. Methods: This retrospective study included 18 children with newly diagnosed aggressive mature B-cell lymphoma after liver transplantation and treated from June 2018 to June 2022 in the Department of Hematology and Oncology of Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine. Clinical characteristics, treatment and outcomes of patients at last evaluation were analyzed. Overall survival (OS) and event free survival (EFS) rates were calculated by Kaplan-Meier method and Log-Rank analysis was performed to find factors of poor prognosis. Results: Among all 18 patients, there were 6 males and 12 females, and the age of onset was 40 (35, 54) months. The interval from transplant to tumor diagnosis was 21 (17, 35) months and 5 patients had early onset disease (<1 year since transplant). Seventeen patients had abdominal lesions. Diarrhea, vomiting and abdominal masses were the main clinical manifestations. All patients were Epstein-Barr virus (EBV) related posttransplant lymphoproliferative disorders (PTLD). One patient received individualized therapy due to critical sick at diagnosis, and the remaining 17 patients received CP (cyclophosphamide, methylprednisolone plus rituximab) and (or) modified EPOCH (prednisone, etoposide, doxorubicin, vincristine, cyclophosphamide plus rituximab) regimens. Of all 18 patients, 15 cases got complete response, 2 cases got partial response, 1 patient died of severe infection. The 2-year OS and EFS rates of 18 patients were (94±5)% and (83±8)%, respectively. None of age, gender or early onset disease had effect on OS and EFS rates in univariate analysis (all P>0.05). Conclusions: The symptoms of PTLD were atypical. Close surveillance of EBV-DNA for patients after liver transplantation was crucial to early stage PTLD diagnosis. CP or modified EPOCH regimen was efficient for pediatric patients with aggressive mature B cell lymphoma after liver transplantation.
目的： 总结儿童肝移植后侵袭性成熟B细胞淋巴瘤的临床特点、诊治要点及预后因素。 方法： 回顾性病例总结。以2018年6月至2022年6月上海交通大学医学院附属上海儿童医学中心血液肿瘤科收治的病理诊断明确的肝移植后侵袭性成熟B细胞淋巴瘤患儿18例为研究对象，分析临床特点、治疗过程和预后等数据，采用Kaplan-Meier方法计算总生存率（OS）和无事件生存率（EFS），采用Log-Rank检验进行预后因素分析。 结果： 18例患儿中男6例、女12例，年龄40（35，54）月龄，肿瘤发生于肝移植后21（17，35）个月，其中早发型病例（肝移植1年以内）5例。17例患儿存在腹部病灶，主要临床表现为腹泻、呕吐和腹部包块。所有患儿均为EB病毒相关移植后淋巴组织增生性疾病（PTLD）。18例患儿中1例起病危重个体化治疗，17例接受CP方案（环磷酰胺+甲泼尼龙联合利妥昔单抗）和（或）改良EPOCH方案（泼尼松+依托泊苷+阿霉素+长春新碱+环磷酰胺联合利妥昔单抗）。18例患儿中15例完全缓解，2例部分缓解，1例严重感染死亡。18例患儿2年OS和EFS分别为（94±5）%和（83±8）%。单因素分析显示，年龄、性别和早发型病例等对OS和EFS的影响差异均无统计学意义（均P>0.05）。 结论： PTLD临床表现不典型，推荐肝移植后持续监测血浆EB病毒DNA以发现早期PTLD。针对肝移植术后侵袭性成熟B淋巴瘤患儿，CP和（或）改良EPOCH化疗方案效果较好。.