Treatment of Children & Adolescents with Early Stage Nodular Lymphocyte Predominant Hodgkin Lymphoma with a Low Intensity Short Duration Chemotherapy Regimen [CVP] - on Behalf of the EuroNet-PHL Group.

Background: Nodular Lymphocyte Predominant Hodgkin’s Lymphoma (NLPHL) is a distinct clinicopathologic subtype of Hodgkin’s lymphoma [HL]. Reports in published literature suggest that very little, and occasionally, no chemotherapy is sufficient for long term survival in patients with LPHL and that this disease appears to have a more indolent course than classical HL. The optimum therapy for early stage NLPHL is not known, but it is likely that current therapy for classical Hodgkin’s lymphoma is unnecessarily toxic and intensive for this indolent, slowly progressive, CD 20 positive disease. Adverse treatment related late effects are the major causes of morbidity and death. Consequently children with early stage NLPHL represent ideal candidates for low intensity treatment.

Study Objectives: To assess whether a non-intensive chemotherapy regimen consisting of cyclophosphamide [500mg/m2 iv- day 1], vinblastine [6 mg/m2 iv-day1 & 8] and oral prednisolone [40 mg/m2 - days 1–7] (CVP) every 14–21 days, count dependent, could replace standard chemotherapy protocols used for classical HL without compromising efficacy in children and young people with NLPHL.

Patients and Methods: Between May 2004 and April 2006 18 patients with stages IA [n=12] and IIA [n=6] biopsy proven NLPHL were treated with 3 cycles of CVP chemotherapy. Three of the eighteen patients were initially treated with surgical resection alone and received CVP at first relapse. Ten patients were males and the median age at diagnosis was 10 years (range 7–15 years). Staging investigations at diagnosis included both conventional cross sectional as well as 18 fluro-deoxyglucose [FDG] PET imaging. Remission status at the end of 3 cycles of CVP was confirmed by both conventional cross sectional and PET imaging.

Results: 17 patients achieved a complete remission [CR] after 3 courses of CVP and 1 patient a very good partial remission [VGPR]. To date, only 1 patient has relapsed while all the remaining patients remain in continuous CR. Median duration of follow up is 12 months (range 2–26 months). The overall survival is 100% and event free survival is 94%. No significant early or late toxicity has been observed to date.

Conclusions: Nothithstanding the relatively short follow up period, CVP is an effective non toxic chemotherapy regimen with 95% of patients with early stage NLPHL achieving a CR after 3 courses. Based on these preliminary results, we have designed a prospective European study for patients with stage IA & IIA NLPHL to confirm these results in a larger cohort of patients.