Disseminated lymphoblastic lymphoma in children and adolescents: results of the COG A5971 trial: a report from the Children%26apos;s Oncology Group

作者：Termuhlen Amanda M^*; Smith Lynette M; Perkins Sherrie L; Lones Mark; Finlay Jonathan L; Weinstein Howard; Gross Thomas G; Abromowitch Minnie

来源：British Journal of Haematology, 2013, 162(6): 792-801.

DOI：10.1111/bjh.12460

• paediatric
• lymphoblastic lymphoma
• non-Hodgkin lymphoma

The Children%26apos;s Oncology Group%26apos;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 2x2 factorial design, the study randomized patients to Children%26apos;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 12%. Age %26lt;10years and institutional imaging response at 2weeks was associated with improved outcomes (P%26lt;0001 and P=0014 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.

• 出版日期2013-9