Burkitt lymphoma is an aggressive form of lymphoma that affects the B-lymphocytes . It accounts for approximately 0.3-1.3% of all non-Hodgkin lymphomas.
Burkitt lymphoma is relatively rare in Western countries, but is quite common in Central Africa. It is named after Dennis Burkitt who first described this kind of tumour in children whilst working as a surgeon in the tropical regions of Africa in 1958. There are three types; endemic, sporadic and immunodeficiency-associated.
How does Burkitt lymphoma affect the body?
Burkitt lymphoma presents with rapidly enlarging lymph node tumour masses involving the chest and/or the abdomen. It has a high tendency to spread to the central nervous system (brain and spinal cord), and can also involve the liver, spleen and bone marrow. Other symptoms that may be present include night sweats, fevers, and unexplained weight loss.
Who does Burkitt lymphoma commonly affect?
Burkitt lymphoma accounts for only 1% of adult lymphoma, but up to 30% of childhood non-Hodgkin lymphomas. The average age of children diagnosed with Burkitt lymphoma is between 5 and 10 years of age, whilst adults are usually diagnosed between the ages of 30 and 50 years. It is 4 times more common in men than in women.
Do we know what causes Burkitt lymphoma?
Burkitt lymphoma, particularly the endemic form that is common in Africa, is associated with the Epstein-Barr Virus (EBV) in nearly 100% of cases. In the sporadic forms that occur in Western countries, EBV is present in approximately 30% of cases and in 40% of immunodeficiency-associated cases. The immunodeficiency-associated form occurs in people who are HIV positive, have AIDS, or are taking immunosuppressive drugs after having an organ transplant. Burkitt lymphoma is usually associated with a mutation involving chromosome 8, affecting a transcription factor known as ‘c-MYC’ which controls many aspects of cell growth. This mutation usually arises by way of a swapping of genes (translocation) between a part of chromosome 8 and a part of one of either chromosome 14, 2 or 22.
How is Burkitt lymphoma treated?
Untreated Burkitt lymphoma has a very aggressive course; however with modern combination chemotherapy regimens, the response rate is very high.
The best and most lasting responses are produced by regimens such as CODOX-M+/-IVAC, HyperCVAD and dose adjusted EPOCH (etoposide, vincristine, doxorubicin, cyclophosphamide and prednisolone). Intrathecal chemotherapy (usually methotrexate and/or cytarabine injected directly into the spine) is also given to prevent or treat Burkitt lymphoma in the central nervous system. The monoclonal antibody rituximab may also be given in combination with chemotherapy in selected situations. Preventing treatment related complications such as tumour lysis syndrome is also very important when treating Burkitt lymphoma.
When appropriate, people will be offered a stem cell transplant - either using their own stem cells (autologous) or those from a donor (allogeneic). New treatments for Burkitt lymphoma are being researched all the time and people may be invited to take part in a clinical trial to assess a new treatment option.