Burkitt lymphoma/leukaemia is the most common (40%) form of
non-Hodgkin lymphoma that occurs in children and adolescents. The prognosis of advanced (disseminated)
Burkitt lymphoma/leukaemia in children and adolescents three decades ago had a 5-year event-free survival (EFS) of <40%, and required
combination chemotherapy and
radiation therapy over a 1-2 year period. Currently, the prognosis for the same advanced stage has a 5-year EFS of 85-90% with <6 months of
chemotherapy only.
Radiation therapy has been eliminated for children and adolescents with
Burkitt lymphoma/leukaemia except in emergencies, such as
superior vena cava syndrome and acute neurological impairment or in patients with relapse/progression. Current risk factors in the prognosis of childhood and adolescent
Burkitt lymphoma/leukaemia include:
lactate dehydrogenase level ≥ 2× the upper normal limit at diagnosis, bone marrow and central nervous system involvement, poor response to
cyclophosphamide,
vincristine and
prednisone reduction
therapy and poor risk cytogenetics. New and novel therapeutic approaches include
monoclonal antibody (anti-CD20)
therapy, targeted cellular immune
therapy and small molecule inhibitors. Future strategies should include improved staging and risk classification, reduction of cytotoxic
chemotherapy, the investigation of targeted
therapy, an increased understanding of the underlying biology of
Burkitt lymphoma/leukaemia, strategies for prevention and approaches to reduce acute and chronic toxicities.