Burkitt Lymphoma · What Is It, Causes, Diagnosis, Treatment, and More

Burkitt lymphoma is a rare but highly aggressive form of non-Hodgkin lymphoma (NHL), a type of blood cancer derived from a group of white blood cells called lymphocytes.  

All lymphomas originate from uncontrolled proliferation of lymphocytes, specifically B-cells and T-cells, which are mainly found in lymph nodes and circulate through the blood and lymphatic system. Generally speaking, lymphomas can be categorized into two main groups: Hodgkin lymphoma and non-Hodgkin lymphoma, where “non-Hodgkin” refers to the absence of Reed-Sternberg cells, which are characteristic of Hodgkin lymphoma.  

Most cases of Burkitt lymphoma are caused by a chromosomal translocation between chromosomes 8 and 14. More specifically, a portion of chromosome 8 containing the MYC oncogene is exchanged for a portion of chromosome 14 that is next to a promoter region (i.e., a region where transcription of the downstream gene is initiated), resulting in overexpression of MYC. Because the MYC gene stimulates cell growth and metabolism, this translocation results in uncontrolled cell division. 

Three types of Burkitt lymphoma exist: endemic, sporadic, and immunodeficiency-related Burkitt lymphoma. Endemic Burkitt lymphoma is one of the most common childhood cancers in equatorial Africa and is almost always associated with Epstein-Barr virus (EBV) infection. EBV is a common and highly contagious virus that is primarily transmitted through the saliva. EBV can infect B lymphocytes and incorporate its genetic material into the host cell’s DNA. While how EBV infection causes lymphoma is complex and still not completely understood, the virus appears to promote cell survival in lymphocytes carrying a MYC translocation.  

On the other hand, sporadic Burkitt lymphoma can occur in both children and adults worldwide and is the most common subtype in the United States and Western Europe. Unlike the endemic form, it is less commonly associated with EBV infection.  

Finally, immunodeficiency-related Burkitt lymphoma usually affects individuals with human immunodeficiency virus (HIV) infection but may also occur in people who take immunosuppressive medications (e.g., corticosteroids, cyclosporin, tacrolimus) to prevent organ transplant rejection.  

Burkitt lymphoma can present with symptoms such as low-grade fever, night sweats, and unintentional weight loss. Most lymphomas also cause lymph nodes enlargement, leading to palpable lumps in different areas of the body, including the neck, armpit, or groin area.  

In addition, Burkitt lymphoma also typically presents extranodal involvement, meaning it can affect other parts of the body in addition to lymph nodes. For example, individuals with endemic Burkitt lymphoma classically present with fast-growing, painless, jaw tumors. On the other hand, sporadic Burkitt lymphoma is characterized by extranodal involvement of the abdomen, most often at the ileocecal junction, a part of the small bowel rich in lymphoid tissue.  

Burkitt lymphoma can also spread to the brain and spinal cord, especially in individuals with the immunodeficiency-related subtype, which can result in headaches, vomiting, altered level of consciousness, and weakness or loss of sensation.  

If the bone marrow is involved, cancer cells can crowd bone marrow progenitor cells and decrease the production of healthy red blood cells, white blood cells, and platelets, causing symptoms like fatigue, recurrent infections, or easy bruising or bleeding. 

Finally, individuals diagnosed with Burkitt lymphoma are at risk of developing tumor lysis syndrome, a condition that occurs when a large number of cancer cells die in a short period of time, releasing a massive amount of breakdown products into the blood. It most commonly occurs within a week of starting cancer treatment, when the tumor burden is higher and cancer cells begin to die. Tumor lysis syndrome can affect various organs, causing symptoms like nausea and vomiting, muscle cramps, dark urine or decreased urine output, and heart palpitations.  

Treatment for Burkitt lymphoma depends on various factors, including the age of the individual, stage, and prognostic features of the cancer. Generally, treatment involves short-course intensive chemotherapy regimens in combination with rituximab, a monoclonal antibody that binds to the surface of B-cells inducing cell death. In individuals with HIV, antiretroviral therapy (ART) is indicated in addition to chemotherapy.  

Despite its aggressive nature, Burkitt lymphoma usually responds well to chemotherapy and has a high cure rate. Involvement of the central nervous system is one of the main risk factors for relapse and usually is associated with a worse prognosis. For this reason, most treatment regimens also include intrathecal chemotherapy, in which the drug is injected directly into the cerebrospinal fluid. For refractory or relapsed cases of Burkitt lymphoma, typically associated with a poor prognosis, a bone marrow transplant from a healthy donor may be used to slow down the progression of the disease. 

Finally, individuals with Burkitt lymphoma may receive supportive treatment to prevent tumor lysis syndrome during chemotherapy. Medications like allopurinol and rasburicase may be useful to help reduce uric acid levels in the blood, and intravenous fluids may be administered to help flush toxins into the urine.  

Burkitt lymphoma, a rare but highly aggressive form of non-Hodgkin lymphoma, is caused by overexpression of an oncogene called MYC, which stimulates uncontrolled cell division. Three types of Burkitt lymphoma exist: endemic Burkitt lymphoma, associated with EBV, affects young children in equatorial Africa; sporadic Burkitt lymphoma occurs in both children and adults worldwide; and immunodeficiency-related Burkitt lymphoma affects immunosuppressed individuals. Burkitt lymphoma classically causes extranodal involvement of the jaw, bones of the face, abdomen, and sometimes, the brain and spinal cord. Diagnosis of Burkitt lymphoma is confirmed with a lymph node biopsy while imaging studies are typically conducted to stage the disease. Treatment involves intensive chemotherapy regimens along with rituximab. Despite its aggressive nature, Burkitt lymphoma responds well to chemotherapy resulting in high cure rates.