Burkitt's lymphoma in Europe

Burkitt's lymphoma (BL) is the most frequent childhood non-Hodgkin's malignant lymphoma (NHML) in Europe and accounts for 5 to 10% of adult NHML. Age distribution is similar to that of endemic BL, with a male:female ratio of 3.7:1. Epstein-Barr virus (EBV) association is found in 15% of cases. A better definition of this monoclonal B-cell malignant proliferation is cytogenetic (i.e., 8;14 or variant translocation). Abdominal masses are initially present in 70% of cases, whereas the jaw is involved in only 4%. The disease is characterized by its overwhelming evolution in the absence of therapy. However, complete remission is usually obtained after the first chemotherapy regimen. In the past, death has been related to cerebrospinal fluid involvement, local recurrence or secondary marrow involvement. Today, it is expected that more than 80% of BL cases will be cured, i.e., 100% of stages I and II abdominal, 70% of stage II non-abdominal and stage III, and 50% of stage IV. Ninety percent of patients alive with no evidence of disease eight months after complete remission can be considered as definitively cured.