Abstract
PurposeTo evaluate the clinical characteristics and treatment outcomes of Chinese children with advanced stage anaplastic large cell lymphoma (ALCL) who were treated with the low-intensity APO regimen.MethodsClinical data from children newly diagnosed with advanced stage ALCL and treated with the APO regimen were reviewed.ResultsAltogether 22 eligible patients with advanced stage ALCL were recruited in this study. 18 (81%) patients achieved complete response (CR) after the initial induction, and 4 experienced relapse. Among patients with relapsed or refractory ALCL, CR was achieved in 3 (50%) who received the BFM95 R3/R4 regimen. Besides, 2 patients received the targeted therapy with crizotinib and were still alive. The 5-year OS and EFS rates were 82 ± 8.7% and 68.2 ± 9.4%%, respectively. According to our results, the elevated LDH level and bone marrow involvement were identified as the poor prognostic factors for EFS (p=0.035 and 0.048, respectively). During APO treatment, only 23% patients experienced grade 3-4 hematologic toxicity.ConclusionsIn this study, bone marrow involvement and elevated serum LDH levels were identified as the poor prognostic factors for EFS. In resource-limited regions, patients with advanced stage ALCL can also achieve comparable outcomes to those in high-income regions, and the BFM95 R3/R4 regimen can take the role of salvage treatment for patients with relapsed or refractory disease. Nonetheless, new therapeutic strategy is still needed.
Highlights
Anaplastic large cell lymphoma (ALCL) is a rare disease in children, which accounts for 10%-15% of all childhood nonHodgkin’s lymphomas [1]
The optimal treatment for pediatric advanced stage ALCL has not been established in terms of its efficacy and safety, especially for patients with relapsed or refractory disease
22 eligible patients with advanced ALCL were recruited into this study, with a median age of 12.5 years at diagnosis
Summary
Anaplastic large cell lymphoma (ALCL) is a rare disease in children, which accounts for 10%-15% of all childhood nonHodgkin’s lymphomas [1]. Despite the variations in regimens, the event-free survival (EFS) in ALCL children is highly consistent across different pediatric studies, with a 2year failure rate of approximately 20-30% [4–6]. In this regard, it is necessary to consider the compliance and availability of a therapeutic regimen, especially in resource-limited regions. This study was designed to evaluate the clinical characteristics and outcomes of Chinese children with advanced stage ALCL who were treated with the low-intensity APO regimen at our institute
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