Burkitt lymphoma

Pronounced BER-kit lim-FOH-muh, Burkitt lymphoma is an aggressive (fast-growing) type of B-cell non-Hodgkin lymphoma that occurs most often in children and young adults.

Large facial Burkitt's Lymphoma

Areas affected

The disease may affect the jaw, central nervous system, bowel, kidneys, ovaries, or other organs.

Also known as small, noncleaved-cell lymphoma. A type of non-Hodgkin’s lymphoma. Burkitt lymphoma (BL) is a very fast-growing type of cancer. It is a form of B-cell non-Hodgkin's lymphoma.

Types

There are three types of Burkitt’s lymphoma, one type occurs in people with weakened immune systems, such as those with AIDS.

Burkitt Lymphoma (FNA, Giemsa stain)

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

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.

Karyotype- burkitt's lymphoma

Cause

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.

Prognosis

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. 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.[8]

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

Inheritance

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.

Burkitt's lymphoma illustration

Diagnosis

Making a diagnosis for a genetic or rare disease can often be challenging. Healthcare professionals typically look at a person’s medical history, symptoms, physical exam, and laboratory test results in order to make a diagnosis. The following resources provide information relating to diagnosis and testing for this condition. If you have questions about getting a diagnosis, you should contact a healthcare professional.

Testing Resources

The Genetic Testing Registry (GTR) provides information about the genetic tests for this condition. The intended audience for the GTR is health care providers and researchers. Patients and consumers with specific questions about a genetic test should contact a health care provider or a genetics professional.

See Also

Non-Hodgkin’s Lymphoma (NHL)

Burkitt lymphoma Resources
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