Promising outcomes of R-CHOP therapy in pediatric nodular lymphocyte-predominant Hodgkin lymphoma: perspectives from a rare subtype.
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BACKGROUND: Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) is a rare sub-type of Hodgkin lymphoma (HL). Due to its rarity, the standard of care for these patients remains poorly defined. Our cohort aimed to evaluate the outcomes of pediatric NLPHL patients treated with the R-CHOP regimen throughout 8 years' single center experience. METHODS: A retrospective cohort study including pediatric patients aged <18 years diagnosed with NLPHL and treated at the Children Cancer Hospital Egypt, between July 2014 and June 2022. RESULTS: In total, 66 patients were included. Twelve patients (18%) with stage IA underwent surgical excision alone. Four of these patients experienced disease progression but achieved a complete response after receiving R-CHOP. Fifty-four patients received R-CHOP as first-line therapy, with early-stage disease received 3-4 cycles, while those with advanced-stage disease received 6 cycles. For this group, the 5-year overall (OS) and event free survival (EFS) were 100% and 91.5%, respectively. The 5-year EFS was not significantly affected by the variant histology pattern among patients received R-CHOP. Patients with stage 3 peripheral disease without risk factors demonstrated outcomes comparable to those with early-stage disease and no risk factors, achieving an excellent 5-year EFS rate of 100%. No significant toxicity was reported, except for grade 3-4 neutropenia. CONCLUSION: R-CHOP is an effective and well-tolerated regimen for treating pediatric NLPHL, demonstrating high OS and EFS rates. Patients with early stage disease achieved particularly favorable outcomes, supporting consideration of treatment de-escalation in this subgroup.