ABSTRACT Background B‐cell lymphoblastic lymphoma (B‐LBL) represents a rare variety of non‐Hodgkin lymphoma, with limited research on its biology, progression, and management. Methods A retrospective analysis was performed on the clinical characteristics of 256 patients aged ≤18 years who received treatment under the China Net Childhood Lymphoma (CNCL)‐NHL‐2017‐lymphoblastic lymphoma regimen from April 2017 to March 2023. Results Among the 256 patients, the median age at diagnosis was 5.0 years, with a slight male predominance. Subcutaneous tissues, skin, and osteolytic bone were the most common sites of the disease. More than 90% of patients exhibited disseminated disease (Stage III or IV). Approximately 19.9% of the diagnosed patients exhibited central nervous system involvement. Adverse events were observed in 33 patients (12.9%), with disease progression or relapse occurring in 10.2% of cases, particularly linked to unfavorable outcomes in instances of early relapse or progression. Salvage chemotherapy combined with immunotherapies, followed by bridging hematopoietic stem cell transplantation, significantly improved the prognosis of relapse and disease progression in children. Overall, the follow‐up time was 37.6 (Q1–Q3, 28.9–38.0) months, and 3‐year event‐free survival rate and overall survivals were 86.3% ± 2.5% and 95.2% ± 1.5%, respectively, with a treatment‐related mortality of 1.6%. Multivariate analysis showed that poor prednisone response and no complete remission on Day 33 of induction were risk factors for poor prognosis. Conclusion The CNCL‐NHL‐2017‐lymphoblastic lymphoma regimen was effective in children with B‐LBL. The response to initial treatment is vital for improving prognosis in patients with B‐LBL.
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