Treatment of mature B-ALL and high grade B-NHL in children

Burkitt Lymphoma and L3ALL are considered to be different forms of the same disease (B-cell disease). Tumour cells have similar cytological and immunological features and display the same non-random translocation involving c- myc on chromosome 8q24 and the gene of an immunoglobulin chain on chromosome 14, 2 or 22. Treatment outcome has greatly improved over the past 15 years as a result of multicentric national trials, especially in Europe, so that the disease has become curable in the majority of patients. Treatment is based on intensive polychemotherapy of short duration and adapted to tumour burden. The major drugs are cyclophosphamide, high-dose methotrexate and cytosine-arabinosine. CNS-directed therapy is essential. Supportive care is also important for the management of the acute treatment-related toxicity. A patient who remains for 1 year in complete remission can be considered as cured, because all relapses occur early within the first year after diagnosis.