Rituximab with chemotherapy in children and adolescents with central nervous system and/or bone marrow-positive Burkitt lymphoma/leukaemia: a childrens oncology group report

Approximately 1 in 4 children and adolescents with de-novo mature and Burkitt lymphoma (BL) present with high-risk disease that is either mature B-cell leukaemia (bone marrow ≥ 25% blasts [BM]) and/or have central nervous system (CNS) involvement. Both the Berlin-Frankfurt- Münster (BFM) and French-American-British (FAB) international cooperative studies have unsuccessfully attempted to reduce the overall burden of chemotherapy in this high risk group of patients. In the FAB 96 study, a randomized attempt to reduce the dose of cytarabine during consolidation and eliminate three final cycles of maintenance was halted early due to inferior event-free survival (EFS) (Cairo, et al 2012, Cairo, et al concluded that reducing the infusion duration of methotrexate from 24 to 4 hours led to significantly inferior EFS in high risk (R3/R4) patients.(Woessmann, et al 2005) Subsets of children with BL, such as those with poor response to initial reduction, complex karyotypes, and those with combined BM and CNS disease, have a significantly worse prognosis (Cairo, et al 2012, Cairo, et al 2007, Poirel, et al 2009).