Short-pulse B-non-Hodgkin lymphoma-type chemotherapy is efficacious treatment for pediatric anaplastic large cell lymphoma: a report of the Berlin-Frankfurt-Munster Group Trial NHL-BFM 90

Anaplastic large-cell lymphoma (ALCL) accounts for approximately 10% of ped iatric non-Hodgkin lymphoma (NHL), Previous experience from NHL-Berlin-Fran kfurt-Munster (BFM) trials indicated that the short-pulse B-NHL-type treatm ent strategy may also be efficacious for ALCL, The purpose of this study wa s to test the efficacy of this protocol for treatment of childhood ALCL in a large prospective multicentertrial and to define risk factors. From April 1990 to March 1995, 89 patients younger than 18 years of age with newly di agnosed ALCL were enrolled in trial NHL-BFM 90, Immunophenotype was T-cell in 40 patients, B-cell in 5, null in 31, and not determined in 13, Stages w ere as follows: I, n = 8; II, n = 20; III, n = 55; IV, n = 6, Extranodal ma nifestations were as follows: mediastinum, n = 28; lung, n = 13; skin, n = 16; soft tissue, n = 13; bone, n = 14; central nervous system, n = 1; bone marrow, n = 5. After a cytoreductive prephase, treatment was stratified int o 3 branches: patients in K1 (stage I and II resected) received three B-day courses (methotrexate [MTX] 0.5 g/m(2), dexamethasone, oxazaphorins, etopo side, cytarabine, doxorubicin, and intrathecal therapy); patients in K2 (st age II nonresected and stage III) received 6 courses; patients in K3 (stage IV or multifocal bone disease) received 6 intensified courses including MT X 5 g/m(2), high-dose cytarabine/etoposide. The Kaplan-Meier estimate for a B-year event-free survival was 76% +/- 5% (median follow-up, 5.6 years) fo r all patients and 100%, 73% +/- 6%, and 79% +/- 11% for K1, K2, and K3, re spectively. Events were as follows: progression during therapy, n = 2; prog ression or relapse after therapy, n = 20; second malignancy, h = 1. It was concluded that short-pulse chemotherapy, stratified according to stage, is effective treatment for pediatric ALCL, B symptoms were associated with inc reased risk of failure. (C) 2001 by The American Society of Hematology.