Burkitt Lymphoma

Overview

Burkitt lymphoma is a fast-growing type of B-cell non-Hodgkin’s lymphoma. Although it grows quickly, patients who receive prompt and effective treatment often experience good outcomes.

There are three main variants of Burkitt lymphoma:

  1. Endemic: this type is found in areas of the world with widespread malaria (such as Africa), and is usually linked with infection with the Epstein-Barr virus (EBV).
  2. Sporadic: this type occurs in other parts of the world, including the United States. EBV infection is sometimes present, but is much less common than in endemic Burkitt lymphoma.
  3. Immunodeficiency-related: this type is most often linked with human immunodeficiency virus (HIV) infection, but can also occur with other causes of immunodeficiency, such as organ transplantation.

All types of Burkitt lymphoma are characterized changes in certain chromosomes that alter the function of a gene known as MYC. This contributes to the growth of Burkitt lymphoma cells.

Symptoms of Burkitt lymphoma may include weight loss, night sweats, and fever. Sporadic Burkitt lymphoma commonly involves the abdomen, and may cause abdominal pain, swelling, nausea and vomiting, or gastrointestinal bleeding.

Diagnosis

Removal and examination of a sample of tumor tissue is necessary to confirm the diagnosis of Burkitt lymphoma. Patients with suspected Burkitt lymphoma will also undergo a number of other tests and procedures, such as a physical examination, blood tests, imaging with tools such as computed tomography (CT), and evaluation of cerebrospinal fluid and bone marrow.

Treatment

Intensive combination chemotherapy is the mainstay of treatment for Burkitt lymphoma, and several different treatment regimens are available. Treatment often includes the drug RituxanÒ (rituximab), which targets B cells. The chemotherapy regimens that are effective against Burkitt lymphoma can produce severe side effects, and it’s important for patients to receive treatment at a center that has experience with this cancer and that can provide the necessary supportive care.

Because Burkitt lymphoma has a tendency to spread to the central nervous system (the brain and spinal cord), preventive treatment of the central nervous system is important. This preventive treatment may involve chemotherapy drugs that are delivered directly into the spinal canal. This is called intrathecal therapy.

Strategies to Improve Treatment

The development of more effective cancer treatments requires that new and innovative therapies be evaluated with cancer patients. Clinical trials are studies that evaluate the effectiveness of new drugs or treatment strategies. Future progress in the treatment of Burkitt lymphoma will result from the continued evaluation of new treatments in clinical trials.  Identification of new treatments is particularly important for patients who have Burkitt lymphoma that returns (relapses) or that does not respond well to initial treatment.

Treatment approaches being evaluated in clinical trials include stem cell transplantation as well as drugs such as Zevalin® (ibritumomab tiuxetan). Zevalin is a radioimmunotherapeutic. Radioimmunotherapy involves treatment with a radioactive substance linked to a monoclonal antibody. In the case of Zevalin, the antibody recognizes and binds to the CD20 protein found on the surface of B cells. The binding of the antibody delivers the radiation directly to the cancer. Zevalin has been approved for the treatment of patients with certain other types of non-Hodgkin lymphoma.

Patients with newly diagnosed or relapsed Burkitt lymphoma who are interested in participating in a clinical trial may wish to discuss the available options with their physician. Information about ongoing clinical trials is also available through the website of the National Cancer Institute.

Reference: Molyneux EM, Rochford R, Griffin B et al. Burkitt’s lymphoma. Lancet. 2012;379:1234-44.