What is Burkitt’s lymphoma

Burkitt’s lymphoma is cancer of the lymphatic system, which can be further divided into Hodgkin’s lymphoma and non-Hodgkin’s lymphoma (NHL)). Burkitt’s lymphoma is classified under non-Hodgkin’s lymphoma.
Lymphatic system is an important part of our body that helps fight infection. It is composed of the lymph nodes, the thymus (a gland behind the breastbone), bone marrow and spleen, which are connected by tiny lymph vessels.
Lymph is a colourless fluid that circulates in the lymphatic system. It contains lymphocytes which are white blood cells that fight infection. There are 2 types of lymphycytes: T cells and B cells. Burkitt’s lymphoma is a fast-growing non-Hodgkin’s lymphoma that originates from B cells.

Statistics on Burkitt’s lymphoma

Burkitt’s lymphoma is a relatively rare disease in Western countries, but is common in Central Africa.
It makes up 0.3-1.3% of all non-Hodgkin’s Lymphoma. The predicted number of new cases of Burkitt’s lymphoma in Australia is 12 per year.
Although a rare condition in adults, Burkitt’s lymphoma is relatively common in children, making up about 30% of all childhood non-Hodgkin’s lymphoma. It more commonly affects the male, with the male to female ratio being around 2-3 to 1. It is also seen more in AIDS patients.

Risk Factors for Burkitt’s lymphoma

Burkitt’s lymphoma, especially the type present in Africa (the endemic form), is closely associated with a virus called Epstein-Barr Virus (EBV). In the non-endemic form, i.e. those occurring in Western countries, EBV is only shown in fewer than 30% of cases.
Burkitt’s lymphoma is also closely related to malaria, and exposure to EBV in infancy. There is a form called the immunodeficiency-associated form which is common in AIDS patients.
In most Burkitt’s lymphomas, there is a change in the gene called translocation, where a part of the gene is transferred to a another location. The translocations that occur in Burkitt’s lymphoma may be of the [t(8;14)], [t(8;2)] or [t(8;22)] type.

Progression of Burkitt’s lymphoma

Burkitt’s lymphoma is the most aggressive subtype of NHL. Because it grows rapidly, patients may soon experience metabolic disturbances and kidney problems.
Burkitt’s lymphoma has a high tendency to spread to the central nervous system (the brain and spinal cord).
Once burkitt’s lymphoma is suspected, diagnosis and staging must be accomplished propmtly, because any delay can result in undesirable effects on the prognosis.
Burkitt’s lymphoma can be staged (into Stage A, AR, B, C and D) as follows:
Stage A: occurring singly in a site outside the abdomen (belly).
Stage AR: occurring inside the abdomen, with more than 90% of the tumour having been cut out.
Stage B: multiple tumours outside the abdomen.
Stage C: occurring inside the abdomen.
Stage D: occurring inside the abdomen plus one or more tumours outside the abdomen.

How is Burkitt’s lymphoma Diagnosed?

Blood tests will be done for evaluation of blood cells, liver function, kidney function and other substances in the blood.

Prognosis of Burkitt’s lymphoma

Prompt treatment of Burkitt’s lymphoma is essential to ensure a good prognosis.
Children generally have a better outlook than adults. With appropriate treatment of Burkitt’s lymphoma, cure can be achieved in 70 to 80% of children and young adults.
The prognosis of Burkitt’s lymphoma depends on the bulk of disease at the time of diagnosis. Burkitt’s lymphoma Patients with limited (Stage A, AR) disease have an excellent prognosis, with a survival rate greater than 90%. Patients (especially adults) with more advanced disease, particularly bone marrow and CNS involvement, have a worse prognosis, but long-term survival can be achieved with more aggressive chemotherapy.

How is Burkitt’s lymphoma Treated?

Treatment of Burkitt’s lymphoma should begin promptly.
The disease can be curable by chemotherapy. For children with localised disease, single therapy using cyclophosphamide has been curative for 80% of children from Africa. Burkitt’s lymphoma patients with extensive disease need combination chemotherapy.
Preventive therapy for central nervous system involvement is essential.
Burkitt’s lymphoma patients with advanced disease may experience tumour lysis syndrome where there is disturbance to a few substances in the body (e.g. urate, phosphate) which may lead to kidney dysfunction. These Burkitt’s lymphoma patients therefore have to be monitored closely during treatment.

Burkitt’s lymphoma References

[1] Braunwald E et al. Harrison’s principles of internal medicine. 15th ed. New York; McGraw-Hill; 2001.
[2] Lymphoma Support and Research Association Inc. How common is lymphoma [online]. [cited 2005 September 11th]. Available from: URL: http://www.lymphoma.org.au/content/?id=25
[3] Souhaimi et al, editors. Oxford Textbook of Oncology. 2nd ed. Oxford ; New York : Oxford University Press; 2002.

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