儿童肝移植后伯基特淋巴瘤：一项关于临床特征、治疗和结局的单中心研究

Post-liver transplantation-Burkitt lymphoma (PLT-BL) is an aggressive and life-threatening complication after pediatric liver transplantation. The research on the disease characteristics, treatment, and prognosis of pediatric PLT-BL is limited.

A retrospective, observational study was performed to analyze the disease characteristics, treatment, and outcomes in 12 pediatric liver transplant (LT) recipients diagnosed with PLT-BL.

The medium time from liver transplantation to diagnosis was 32.71 months (range, 25.78-37.85 months). All patients (100%) tested positive for EBV viremia at diagnosis. Abdomen and peripheral lymph nodes were the most frequently involved sites (11 [91.66%] and 10 [83.33%], respectively). Three pediatric patients were diagnosed as relapsed or refractory PLT-BL and treated with chimeric antigen receptor (CAR) T-cell therapy, and two of them achieved complete remission (CR). Nine patients were alive with CR at the last follow-up. Three deaths were attributed to progression and tumor lysis syndrome, characterized by significantly elevated lactate dehydrogenase (LDH) levels (p = 0.027), more advanced tumor stages (p = 0.045), and an increased number of involved sites (p = 0.009). With a median follow-up of 2.7 years after diagnosis, the event-free survival (EFS) and overall survival (OS) rates for PLT-BL patients were 66.7% and 74.1%, respectively. In four patients, immunosuppression was successfully withdrawn and they maintained tolerance status.

Elevated LDH levels, more advanced tumor stages, and increased number of involved sites are potentially associated with poor outcome. Immunosuppression withdrawal is a safe and feasible approach for PLT-BL.