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Keywords:

  • B-NHL03;
  • childhood;
  • JPLSG;
  • non-Hodgkin lymphoma

Background

Previous Japanese studies of childhood B-cell non-Hodgkin lymphoma (B-NHL) have shown a favorable outcome, though the study size was too small to effectively assess the efficacy and safety of treatment for childhood B-NHL.

Procedure

We performed a nation-wide prospective B-NHL03 study to assess the efficacy and safety of short-pulse intensive chemotherapy for children with B-NHL. They were stratified into four treatment groups according to disease stage, tumor resectability and bone marrow/CNS involvement: Group 1 with all resected stage I/II, Group 2 with non-resected stage I/II, Group 3 with stage III & CNS-negative stage IV, and Group 4 with CNS-positive stage IV & Burkitt leukemia. Treatment duration was 2 courses for Group 1, 4 courses for Group 2, and 6 courses for Groups 3 and 4, respectively. CNS irradiation was omitted in all patients.

Results

The follow-up time ranged from 0.8 to 88 months, with a median of being 45 months. For 321 patients analyzed in this study, overall survival and event-free survival (EFS) at 4 years was 92.7% and 87.4%, respectively. The 4-year EFS according to treatment group were 94% for Group 1 (n = 17), 98% for Group 2 (n = 103), 84% for Group 3 (n = 111), and 78% for Group 4 (n = 90). There was no significant difference in outcome by histology. Therapy-related death occurred in three patients in remission.

Conclusions

Our nationwide large-scale study resulted in a cure rate above 90% with <1% toxic death in childhood B-NHL. Pediatr Blood Cancer 2014;61:1215–1221. © 2014 Wiley Periodicals, Inc.