Post-Liver Transplantation-Burkitt Lymphoma in Children: A Single-Center Study of Clinical Characteristics, Treatment, and Outcomes.
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BACKGROUND: 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. METHODS: 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. RESULTS: 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 In four patients, immunosuppression was successfully withdrawn and they maintained tolerance status. CONCLUSION: 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.