Lymphoma is a cancer of lymphocytes, which are white blood cells of the immune system that circulate in lymph nodes throughout the body and the spleen. Lymphoma is divided into two main subtypes: Hodgkin lymphoma (HL) and Non-Hodgkin Lymphoma (NHL). Within the category of Non-Hodgkin lymphoma, there are many different subtypes. Burkitt lymphoma is a rare subtype of Non-Hodgkin lymphoma and accounts for only ~1% of Non-Hodgkin lymphoma in adults.
There are three main types of Burkitt lymphoma: endemic, sporadic and immunosuppression-related. The endemic form is generally found in sub-Saharan Africa. This form usually presents in the jaw or as a facial bone tumor. The sporadic form usually presents with an intra-abdominal mass with symptoms of bowel obstruction or gastrointestinal bleeding. The immunodeficiency-related case involves lymph nodes and can be seen in connection with HIV infection or after organ transplantation.
Burkitt lymphoma can cause enlarged lymph nodes throughout the body as well as an enlarged liver and spleen. Burkitt lymphoma can affect the bone marrow and the circulating blood. In addition it also can affect the gastrointestinal tract and the brain.
Symptoms of Burkitt Lymphoma
Symptoms for Burkitt lymphoma can be enlarged lymph nodes and/or the spleen. Burkitt lymphoma can also cause fevers, chills, night sweats and unexplained weight loss (B-symptoms). Gastrointestinal symptoms can also be common.
Risk Factors for Burkitt Lymphoma
There are no known inherited predisposition to Burkitt lymphoma. In the endemic type of Burkitt lymphoma, there is a strong association with Epstein-Barr Virus (EBV )infection. In the immunodeficiency type of Burkitt lymphoma, there is a clear association with decreased function of the immune system to destroy early pre-cancerous cells.
How Burkitt Lymphoma is Diagnosed
The diagnosis is usually made by surgical removal of one of the enlarged lymph nodes. Since there are many other lymphomas that can resemble this disease and the treatments are different, it is important to carefully review the lymph node under the microscope. The diagnosis is confirmed by the presence of a certain genetic abnormality, an abnormal chromosome fusion that results in over-expression of the MYC protein in the lymphoma cells. FISH (fluorescence in-situ hybridization) is a test that can detect this chromosome abnormality in the lymphoma cells. Laboratory studies have shown that the MYC protein is responsible for promoting the survival and growth of the cancerous cells.
Once the diagnosis of Burkitt lymhompa is established, computed axial tomography (CAT) scans of the neck, chest, abdomen and pelvis are necessary to determine the degree and extent of involvement of the lymph nodes. A bone marrow biopsy is usually performed to determine the degree of involvement of the bone marrow. Together, these tests establish the clinical stage of the lymphoma.
Flow cytometry analysis of the lymphoma cells will determine whether the cancer cells express certain proteins on their surface that are characteristic of Burkitt lymphoma. Evaluation of the gastrointestinal system for lymphoma involvement is sometimes necessary requiring an endoscopy, particularly if the patient is having any gastrointestinal symptoms. Evaluation of heart function with an echocardiogram or Multiple Gated Acquisition (MUGA) scan can be necessary before treatment with chemotherapy drugs is considered since adriamycin, one of the main drugs used in treatment, can affect heart function at high doses. The brain can also be involved by lymphoma; therefore, it is necessary to sample the fluid surrounding the brain and spinal cord (cerebrospinal fluid) for evidence of lymphoma as well as to administer chemotherapy to the fluid around the brain. This fluid is accessed through a procedure called a lumbar puncture or “spinal tap.”
Treatment Options for Burkitt Lymphoma at Tufts Medical Center
There are several different regimens for treatment of Burkitt lymphoma. All treatments are intensive, which reflect the aggressive nature of this lymphoma. One common chemotherapy regimen is HyperCVAD (hyperfractionated) cyclophosphamide, Vincristine, doxorubicin, and dexamethasone, alternating with high-dose methotrexate and cytarabine). The monoclonal antibody rituximab may also be given depending on the clinical situation. This regimen requires staying in the hospital since the intravenous medications are given continuously. Stem cell transplantation can also be part of the treatment sequence depending on how aggressive the lymphoma is, the age and the condition of the patient.
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Research + Clinical Trials
The purpose of this study is to evaluate the effects, good and bad of a new drug called ixazomib (also called MLN9708), when it is given along with a common treatment combination, called Dose-Adjusted EPOCH-R (DA-EPOCH-R, for short). This is a type of study called a phase I/II trial. In the phase I part, the dose of the study drug (ixazomib) will be adjusted (either up or down) to find the maximum (highest) dose that does not cause excessive (too many) harmful side effects. In the phase II part, this dose of ixazomib will be given at the maximum safe dose found in phase I. In both phase I and II, DA-EPOCH-R will be adjusted between cycles depending on how blood cell levels are affected between cycles. Ixazomib is considered investigational because it is not approved by the U.S. Food and Drug Administration (FDA). DA-EPOCH-R is a combination chemotherapy treatment developed over the last 14-15 years, and each of the drugs in this regimen is FDA-approved and considered part of the standard of care.
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