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DOI: 10.1055/s-0034-1371120
Treatment of Pediatric Lymphocyte Predominant Hodgkin Lymphoma (LPHL): A Report from the Children's Oncology Group
Question: LPHL typically presents with low stage disease and responds to classical HL regimens. Recurrence is uncommon, but second malignancies including non-Hodgkin lymphoma (NHL) can occur. Thus, reducing radiation exposure may be of benefit. We report the results of a prospective trial in which a selected subset of patients had surgery alone and the rest were treated with limited chemotherapy ± involved-field radiation therapy (IFRT).
Methods: Patients < 21 years with newly diagnosed, low risk LPHL were eligible for AHOD03P1. Low risk was defined as clinical Stage IA or IIA without bulk disease (mediastinal mass > 1/3 of the thoracic diameter or nodal aggregate > 6 cm). Patients with Stage IA with an unresected node, more than a single involved node or Stage IIA were treated with 3 cycles of AV-PC (doxorubicin/vincristine/prednisone/cyclophosphamide). Patients with Stage IA in a single node that was completely resected were initially observed without further therapy; those who recurred after surgery with low risk LPHL received AV-PC x 3. Patients with < complete response (CR) to AV-PC x 3 received 2100 cGY IFRT.
Results: 185 eligible patients with low risk LPHL were enrolled; 180 completed protocol therapy.
52 patients had initial surgery alone. There were 12 relapses at a median of 11.3 months; 4 year EFS = 79%.
137 patients received AV-PC x 3; 128 were treated at diagnosis and 9 at relapse after surgery alone. 11 patients receiving AV-PC had < CR and received IFRT.
14 first events occurred among these 137 patients (12 relapses and 2 NHL). One relapse occurred in a patient who received IFRT. Current 4-year EFS estimate is 86.8% for these 137 patients.
4 year EFS for the entire cohort of 185 patients = 83.7%. The median follow-up time among 158 patients without events is 43.6 (range 0.03 – 86.0) months; OS is 100%.
Conclusions: Pediatric LPHL has an excellent EFS with chemotherapy that is less intensive than standard regimens; > 90% of patients can avoid RT. NHL as a first event may be related to LPHL and not an effect of treatment. The salvage rate for the few relapses is high, with 100% survival of the entire cohort.