Burkitt lymphoma (BL) is a very fast-growing type of cancer. It is a form of B-cell non-Hodgkin's lymphoma. There are 3 recognized forms of BL:

• Endemic (African) - the most common form, found mainly in central Africa, where it is associated with the Epstein Barr virus (EBV). It is most common in children. This form often manifests as enlargement of the jaw or facial bones.
• Sporadic - a rarer form, seen in all parts of the world, that often develops in the abdomen with bone marrow involvement. The kidneys, ovaries, breasts or other organs may also be involved. This form commonly affects children and young adults.
• Immunodeficiency-associated - occurs primarily in people with HIV infection, and less commonly in people with other immunodeficiency disorders or recipients of organ transplants.

Signs and symptoms may differ depending on the form of BL and the organs or body systems involved. When it spreads, weakness and fatigue often develop. Lymphoma cells may build up in the lymph nodes and other organs, causing swelling. Central nervous system involvement is possible with all forms of BL, particularly when there is advanced-stage disease.

The exact cause of BL is not known. EBV infection appears to play a role in virtually all cases of endemic (African) BL, and a minority of sporadic and immunodeficiency-associated BL. While acquired (not inherited) genetic changes involving the MYC gene and other genes are present within BL cancer cells, it is unclear what causes these genetic changes to occur.Without timely treatment, BL is rapidly fatal. Treatment involves intensive chemotherapy, which includes chemotherapy to the fluid surrounding the brain and spinal cord. The majority of people treated with aggressive therapy achieve long-term remission.

Burkitt lymphoma (BL) is not an inherited condition. It almost always occurs in people with no family history of BL. To our knowledge, there has been one report (in 1986) describing BL in more than one family member (two sisters). However, this occurrence was thought to be due to an inherited lymphocyte disorder that may have predisposed the sisters to developing BL.

While BL is associated with genetic changes involving the MYC gene and immunoglobulin genes (genes that provide instructions for antibodies), these genetic changes are acquired (not inherited), and are limited to the cancer cells. They are not passed on to offspring.

Treatment options for newly diagnosed Burkitt and Burkitt-like lymphoma/leukemia

Treatment options for newly diagnosed Burkitt and Burkitt-like lymphoma /leukemia may include:

• Surgery to remove as much of the tumor as possible, followed by combination chemotherapy.
• Combination chemotherapy with or without targeted therapy (rituximab).

Treatment options for recurrent Burkitt and Burkitt-like lymphoma/leukemia

Treatment options for recurrent Burkitt and Burkitt-like non-Hodgkin lymphoma /leukemia may include:

• Combination chemotherapy with or without targeted therapy (rituximab).
• High-dose chemotherapy with stem cell transplant with the patient's own cells or cells from a donor.
• Targeted therapy with a bispecific antibody.
• A clinical trial that checks a sample of the patient's tumor for certain gene changes. The type of targeted therapy that will be given to the patient depends on the type of gene change.

We are not aware of statistics regarding the recurrence rate (or risk of relapse) of Burkitt lymphoma (BL) in general. There have been studies that have "lumped together" both relapsing and refractory cases (those that are resistant to treatment). Depending on the study, the combined incidence of these cases has ranged from about 1 in 7 people (15.2%) to 1 in 16 people (6.4%).

The majority of relapses occur during the first year of treatment for BL. Therefore, people who remain disease-free at 10-12 months are considered cured, although reports of delayed relapses (even several or more years later) have been described.

Due to the risk of recurrence, it is typically recommended that individuals be monitored at least every 2 months during the first year after chemotherapy is completed. They should additionally be monitored every 3 months the following year, and every 6 months thereafter.

Without timely treatment, BL is rapidly fatal. However, the majority of people treated with aggressive therapy achieve long-term remission.

In general, children with BL have better survival rates than adults with BL. The prognosis in children correlates with the extent of disease at the time of diagnosis. Those with limited disease when diagnosed and treated have a survival rate greater than 90%. Children with more extensive disease, especially involving the bone marrow and central nervous system, have long-term survival rates of 50-90%.

While the majority of adults with BL also achieve long-term remission with aggressive therapy, adults (particularly those with advanced stage disease) do more poorly than children. In addition to the extent of disease at the time of diagnosis, prognosis depends on the age of the adult. In general, survival rates decrease with age and are lowest for elderly patients. Unfortunately, specific statistics regarding the prognosis for adults with BL appear to be more scarce.

The outlook is poor for both children and adults if BL returns after improvement (relapses) or does not go into remission as a result of the first cycle of chemotherapy.