Close
Close

Burkitt's lymphoma

Burkitt lymphoma, BL

Overview

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.

It is named after Denis Parsons Burkitt, a surgeon who first described the disease in 1956 while working in equatorial Africa. This type of tumor was first discovered in children in certain parts of Africa, but it also occurs in the United States. The African type of Burkitt lymphoma is closely associated with the Epstein-Barr virus (EBV), the main cause of infectious mononucleosis. The North American form of Burkitt lymphoma is not linked to EBV. The majority of Burkitt lymphoma cases are seen in males.

Symptoms - Burkitt's lymphoma

Burkitt lymphoma may first be noticed as a swelling of the lymph nodes (glands) in the neck, groin, below the jaw, or under the arm. These swollen lymph nodes are often painless, but can grow very rapidly. In the more common types seen in the United States, the cancer usually starts in the belly area (abdomen). The disease can also start in the ovaries, testes, brain, and spinal fluid.

Symptoms include:

  • Nodes that grow together to form a lump
  • Non-tender nodes
  • Rapid growth of the lymph nodes
  • Unexplained swollen lymph nodes

Causes - Burkitt's lymphoma

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.

Prevention - Burkitt's lymphoma

Not supplied.

Diagnosis - Burkitt's lymphoma

Healthcare professionals 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.

Prognosis - Burkitt's lymphoma

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.

Treatment - Burkitt's lymphoma

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.

Effect of the chemotherapy, as with all cancers, depends on the time of diagnosis. With faster growing cancers, such as this one, the cancer actually responds faster than with slower growing cancers. This rapid response to chemotherapy can be hazardous to patient as a phenomenon called "tumor lysis syndrome" could occur. Close monitoring of patient and adequate hydration is essential during the process.

Chemotherapy:

  • Cyclophosphamide
  • Doxorubicin
  • Vincristine
  • Methotrexate
  • Cytarabine
  • Ifosfamide
  • Etoposide
  • Rituximab

Other treatments are immunotherapy, bone marrow transplants, surgery to remove the tumor, and radiotherapy

Resources - Burkitt's lymphoma

  • NIH
Orphan Therapies
Videos
by Dr. Jules Richard Kemadjou
8,867 views
Research Publications