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    Childhood Non-Hodgkin Lymphoma Treatment (PDQ®): Treatment - Health Professional Information [NCI] - High-Stage Childhood B-cell NHL Treatment

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    Rituximab is a mouse/human chimeric monoclonal antibody targeting the CD20 antigen. Among the lymphomas that occur in children, diffuse large B-cell lymphoma and Burkitt lymphoma both express high levels of CD20.[6] Rituximab has been safely combined with standard doxorubicin, cyclophosphamide, vincristine, and prednisone (CHOP) chemotherapy and has been shown to improve outcome in a randomized trial of adults with diffuse large B-cell lymphoma (CAN-NCIC-LY9).[7,8] In an adult study, rituximab has also been safely combined with an intensive chemotherapy regimen used to treat patients with Burkitt lymphoma.[9] In children, a single-agent phase II study of rituximab performed by the BFM group showed activity in Burkitt leukemia and lymphoma.[10][Level of evidence: 2Div] A Children's Oncology Group (COG) pilot study (COG-ANHL01P1) added rituximab to baseline chemotherapy with FAB/LMB-96 therapy in patients with stage III and stage IV B-cell NHL; compared with chemotherapy-only protocols, toxicity was similar, despite a trend toward higher peak rituximab levels in younger patients.[11]; [12][Level of evidence: 3iiiA] The addition of rituximab to standard chemotherapy is now being tested in a randomized trial.

    Standard Treatment Options

    Current data do not suggest superiority for either of the following standard treatment options.

    Table 5. Standard Treatment Options for High-Stage B-cell NHL

    Stratum Disease Manifestations Treatment
    ALL = acute lymphoblastic leukemia; BFM = Berlin-Frankfurt-Munster; CNS = central nervous system; LDH = lactate dehydrogenase; NHL= non-Hodgkin lymphoma.
    FAB/LMB-96 International Study COG-C5961 (FAB/LMB-96)[2,3] B Multiple extra-abdominal sites Prephase + four cycles of chemotherapy (reduced intensity arm)[2]
    Nonresected stage I and II, III, IV
    Marrow <25% blasts
    No CNS disease
    C Mature B-cell ALL (>25% blasts in marrow) and/or CNS disease Prephase + eight cycles of chemotherapy (full intensity arm)[3]
    BFM Group[1,5] R2 Nonresected stage I/II and stage III with LDH <500 IU/L Prephase + four cycles of chemotherapy (4 h methotrexate infusion)[1]
    R3 Stage III with LDH 500-999 IU/L Prephase + five cycles of chemotherapy (24 h methotrexate infusion)[1]
    Stage IV, B-cell ALL (>25% blasts) and LDH <1,000 IU/L
    No CNS disease
    R4 Stage III, IV, B-cell ALL with LDH >1,000 IU/L Prephase + six cycles of chemotherapy (24 h methotrexate infusion)[1]
    Any CNS disease
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