Efficacy evaluation of the 2017 protocol by the China Net Childhood Lymphoma in treating early T cell precursors-lymphoblastic lymphoma in pediatric patients.

To analyze the clinical features of early T-cell precursor acute lymphoblastic leukemia/lymphoma (ETP-ALL/LBL) in pediatric patients and to summarize the efficacy evaluation of the 2017 protocol by the China Net Childhood Lymphoma (CNCL) Non-Hodgkin Lymphoma (NHL) 2017-LBL for ETP-LBL. Methods Clinical data of 19 children with ETP-LBL admitted to CNCL from May 2017 to August 2023 were retrospectively collected. Results The median age of onset was 10 years (4.5-13 years), including 16 males (84.2 %). All patients were in stage IV and were treated with high-risk chemotherapy regimen. 11 of them (57.9 %) underwent hematopoietic stem cell transplantation (HSCT). The proportion of leukemia stage in the HSCT group was significantly higher than that in the chemotherapy group (72.7 % vs. 25.0 %), and the central nervous system (CNS) status was CNS2/3 in the HSCT group (100 % vs. 50 %).The 3-year overall survival (OS) and event free survival (EFS) were (93.8 ± 0.61) % and (88.2 ± 0.78) %, respectively. Conclusion Over 50 % of pediatric ETP-LBL patients received HSCT, with their long-term survival rates comparable to the chemotherapy group.