What is Burkitt Lymphoma?
Burkitt Lymphoma is a highly aggressive form of non-Hodgkin lymphoma that originates from
B cells. It is characterized by the rapid growth of tumors in various parts of the body, particularly the lymph nodes, bone marrow, and central nervous system. Named after the British surgeon Denis Burkitt, who first identified the disease in African children in 1958, this lymphoma can be categorized into three main types: endemic, sporadic, and immunodeficiency-associated.
What Causes Burkitt Lymphoma?
The primary cause of Burkitt Lymphoma is the translocation of the
c-MYC gene to a region under control of the immunoglobulin heavy or light chain gene enhancers, leading to uncontrolled cell division. The Epstein-Barr virus (
EBV) is commonly associated with the endemic form of Burkitt Lymphoma, particularly in African children. In contrast, the sporadic form, more common in Western countries, and the immunodeficiency-associated form, often seen in patients with
HIV/AIDS, may not always have a direct association with EBV.
What are the Symptoms?
Symptoms of Burkitt Lymphoma can vary depending on the location of the tumor. Common symptoms include:
Swelling of the lymph nodes
Abdominal pain or fullness
Unexplained weight loss
Night sweats
Fever
Facial swelling or jaw pain (more common in endemic cases)
How is It Diagnosed?
The diagnosis of Burkitt Lymphoma typically involves a combination of physical examination, imaging studies, and laboratory tests. A
biopsy of the affected lymph node or tumor is essential to confirm the diagnosis. Additional tests may include
immunohistochemistry, cytogenetic analysis, and molecular studies to identify the characteristic MYC gene translocation.
PET scans and
MRI may also be used to assess the extent of the disease.
What are the Treatment Options?
Burkitt Lymphoma is treated with intensive chemotherapy regimens, which may include drugs such as cyclophosphamide, doxorubicin, vincristine, and methotrexate. Because of the aggressive nature of the disease, treatment often needs to be initiated quickly. In some cases, targeted therapies like
rituximab, a monoclonal antibody against the CD20 protein on B cells, may be added to the regimen. Supportive care, including the management of tumor lysis syndrome, is crucial during treatment.
What is the Prognosis?
The prognosis for Burkitt Lymphoma varies depending on several factors, including the stage at diagnosis, the patient's age, and overall health. With prompt and aggressive treatment, the prognosis can be favorable, with many patients achieving long-term remission. However, the disease can be fatal if not treated timely and adequately.
