Treatment of advanced stage diffuse, small non‐cleaved cell lymphoma in childhood: Further experience with total therapy B

Prior to the development of intensive treatments for patients with advanced stage B‐cell non‐Hodgkin's lymphoma (B‐NHL), the prognosis for such patients was dismal. A minority of patients attained long‐term, disease‐free survival. Since 1981, we have treated 28 children with advanced stage B‐NHL with an intensive chemotherapeutic protocol, Total Therapy B. This regimen employs cycles of fractionated high‐dose cyclophosphamide, doxorubicin, and vincristine alternating with sequential infusions of high‐dose methotrexate and escalating doses of cytarabine, in addition to intensive intrathecal therapy. The planned duration of therapy is approximately 6 months. Two patients had B‐cell acute lymphoblastic leukemia and 26 had stage III B‐NHL; none had CNS involvement. The median age was 7 years. All 28 patients achieved complete remission (CR). Both patients with B‐ALL and 21 of 26 with stage III B‐NHL remain in CR, with a median follow‐up of 51 months. Treatment failures included 3 patients with recurrent or progressive disease, 1 toxic death in CR, and 1 patient who developed a secondary mediastinal T‐cell lymphoblastic lymphoma 4 1/2 years after the diagnosis of B‐NHL. The 2‐ and 5‐year event‐free survival rates were 85.7 ± 6.6% (SE) and 79.6 ± 8.5%, respectively. Total Therapy B is a highly effective therapy for children with advanced stage B‐NHL without CNS involvement. © 1994 Wiley‐Liss, Inc.