Disseminated lymphoblastic lymphoma in children and adolescents: Results of the COG A5971 trial: A report from the Children's Oncology Group

Amanda M. Termuhlen, Lynette M Smith, Sherrie L. Perkins, Mark Lones, Jonathan L. Finlay, Howard Weinstein, Thomas G. Gross, Minnie Abromowitch

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34 Scopus citations


The Children's Oncology Group's A5971 trial examined central nervous system (CNS) prophylaxis and early intensification in paediatric patients diagnosed with CNS-negative Stage III and IV lymphoblastic lymphoma. Using a 2 × 2 factorial design, the study randomized patients to Children's Cancer Group (CCG) modified Berlin-Frankfurt-Muenster (BFM) acute lymphoblastic leukaemia (ALL) regimen with intensified intrathecal (IT) methotrexate (MTX) (Arm A1) or an adapted non-Hodgkin lymphoma/BFM-95 therapy with high dose MTX in interim maintenance but no IT-MTX in maintenance (Arm B1). Each cohort was randomized ± intensification (cyclophosphamide/anthracycline) (Arms A2/B2). For the 254 randomized patients, there was no difference in 5-year event-free survival (EFS) for the four arms: Arm A1, 80% [95% confidence interval (CI) 67-89%] and Arm A2, 81% (95% CI 69-89%); Arm B1, 80% (95% CI 68-88%) and Arm B2, 84% (95% CI 72-91%). The cumulative incidence of CNS relapse was 1·2%. Age <10 years and institutional imaging response at 2 weeks was associated with improved outcomes (P < 0·001 and P = 0·014 for overall survival). CNS positive patients (n = 12) did poorly [5-year EFS of 63% (95% CI 29-85%)]. For CNS-negative patients, there was no difference in outcome based on CNS prophylaxis (IT-MTX versus HD-MTX) or with intensification.

Original languageEnglish (US)
Pages (from-to)792-801
Number of pages10
JournalBritish Journal of Haematology
Issue number6
Publication statusPublished - Sep 2013



  • Lymphoblastic lymphoma
  • Non-Hodgkin lymphoma
  • Paediatric

ASJC Scopus subject areas

  • Hematology

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