What are the other Names for this Condition? (Also known as/Synonyms)

• Burkitt's Lymphoma
• Burkitt's Tumor
• Malignant Lymphoma, Burkitt's Type

What is Burkitt Lymphoma? (Definition/Background Information)

• Burkitt Lymphoma is a very aggressive and fast-growing form of non-Hodgkin lymphoma. The lymphoma affects the B-cells of an individual’s immune system
• There are 3 different kinds of Burkitt Lymphoma:
  • Endemic: This type occurs in sub-Saharan Africa and is strongly linked to Epstein-Barr virus and chronic malaria. One of the classic symptoms of endemic Burkitt Lymphoma is swelling of the jaw
  • Sporadic: This form is found outside of sub-Saharan Africa. There is some link to the Epstein-Barr virus, but it is not as strong as the endemic form. Abdominal symptoms are more common in this form of Burkitt Lymphoma
  • Immunodeficiency-associated: This form of Burkitt Lymphoma occurs mainly in individuals with HIV/AIDS. However, it can also develop in those who have had organ transplants and are taking immunosuppressants (drugs to reduce the likelihood that the body will reject the transplanted organ)
• The specific cause of this condition is currently unknown; however, genes that likely play a role, have been identified. The most important gene involved is the c-MYC gene
• The gene involvement must be present, to make a valid diagnosis of Burkitt Lymphoma. A diagnosis is typically made with the biopsy of affected lymph nodes or tumor tissue
• Burkitt Lymphoma is generally treated with intensive chemotherapy supplemented with monoclonal antibody treatment (a drug that directly targets B cells)
• As of now, the standard of care is intensive chemotherapy; but, a new clinical study has determined that low intensity chemotherapy may be effective in some cases. This provides the added benefit of less toxicity

Who gets Burkitt Lymphoma? (Age and Sex Distribution)

• Children are more likely to develop Burkitt Lymphoma than adults; young boys around the ages of 5-8 years are especially susceptible to developing this disease
• Around two-thirds of adults with the condition are males
• Individuals living in sub-Saharan Africa are much more likely to suffer the condition, than rest of the world
• Those with HIV/AIDS are also more likely to be affected by Burkitt Lymphoma

What are the Risk Factors for Burkitt Lymphoma? (Predisposing Factors)

The risk factors of Burkitt Lymphoma include:

• Young boys in the age group of 5-8 years and adult males
• Individuals belonging to the endemic regions of sub-Saharan Africa
• Individuals with weak immune systems, such as those having HIV/AIDS, organ transplant recipients who are on immunosuppressant drugs, etc.

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Burkitt Lymphoma? (Etiology)

• The exact cause of Burkitt Lymphoma is unknown; though, genes (most importantly the c-MYC gene) are known to be involved
• In many cases, B-cells have a mutation in the proto-oncogene MYC
• However, it is known that Burkitt Lymphoma is linked to Epstein-Barr virus, chronic malaria, and HIV

What are the Signs and Symptoms of Burkitt Lymphoma?

The signs and symptoms of Burkitt Lymphoma include:

• The main symptom is inflammation of the lymph nodes in the neck, groin, chest, abdomen, or armpit regions. However, the swelling is usually painless
• Enlarged lymph nodes in the chest and neck areas can result in swallowing difficulty, breathlessness, or sore throat
• The lymphoma may also cause fluid to collect in the individual’s abdomen (ascites). Due to this fluid collection, the gastrointestinal (GI) tract may also be affected, causing abdominal pain, diarrhea, and nausea
• Other general symptoms may include:
  • Night sweats
  • Fatigue
  • Loss of appetite
  • Fever
  • Weight loss
• If the cancer has reached the bone marrow, individuals may display anemia (low red blood cell count) and thrombocytopenia (low platelet count)
• Burkitt Lymphoma can also affect the central nervous system. If the cancer has spread to these regions, then the individual may have headaches, confusion, an inability to focus, and seizures
• Some symptoms are specific to the two types of Burkitt Lymphoma - namely the endemic type and sporadic type
  • Endemic type involves swelling of the jaw, while the sporadic form typically does not involve the same
  • Abdominal symptoms are more commonly found in the sporadic form, than the endemic form

How is Burkitt Lymphoma Diagnosed?

A diagnosis of Burkitt Lymphoma may involve:

• A physical examination and evaluation of medical history
• Typically, this cancer is diagnosed by a tumor biopsy - biopsy of an enlarged lymph node. The tissue is looked at under the microscope by a pathologist, in order to make a definitive diagnosis
• Additional testing may be done to aid in the diagnosis, which may include:
  • Blood tests
  • Bone marrow tests
  • Lumbar puncture
  • Lymphangiogram (LAG)
• CT and MRI scans may also be performed, to determine the stage of cancer

There are 4 possible stages of Burkitt Lymphoma:

• Stage I - only one group of lymph nodes has cancer
• Stage II - two groups of lymph nodes are cancerous. However, the two groups are either in the chest area (above the diaphragm) or in the abdomen region (below the diaphragm)
• Stage III - multiple groups of lymph nodes are affected, and the cancer is present above and below the diaphragm
• Stage IV - the lymphoma has reached the bone marrow or organs, not in the lymphatic system

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Burkitt Lymphoma?

Complications of Burkitt Lymphoma are generally the result of chemotherapy, or due to cancer spread. These may include:

• The lymphoma generally spreads to the abdomen, blood, bone marrow, or the central nervous systems. The treatment may be adjusted by the oncologist, to deal with spread of the cancer
• Chemotherapy weakens the immune system, and so an infection may be a possible complication. Chemotherapy may also cause the following:
  • Anemia
  • Thrombocytopenia
  • Ulcers in the mouth
  • Nausea
  • Hair loss
  • Fatigue
  • Peripheral neuropathy (abnormal changes in sensation/feeling in one’s hands and feet)
• Another major complication of the condition is tumor lysis syndrome. The death and breakdown of cancer cells can cause the kidneys to be overloaded. In order to prevent this complication, physicians usually prescribe rasburicase. However, if left untreated, tumor lysis syndrome can result in the need for dialysis

How is Burkitt Lymphoma Treated?

The most common form of treatment of Burkitt Lymphoma is intensive chemotherapy. The treatment usually lasts at least a month, but may also last up to 8-months. An inpatient treatment is provided, due to the fact that some of the drugs need to be given quite frequently, or need to be continually administered.

• Chemotherapy drugs that may be used include: Cyclophosphamide, vincristine, ifosfamide, doxorubicin, methotrexate, cytarabine, and etoposide
• The drugs are usually given intravenously, either through a central line (catheter placed in a vein near the chest/heart) or through a PICC line (peripherally inserted central catheter) in the arm
• Also, chemotherapy may be given intrathecally, to deliver the drugs directly to the cerebrospinal fluid, in order to prevent the spread of the cancer to the fluid, or to treat the condition if cancer has spread to the cerebrospinal fluid
• Rituximab may be used in combination with the chemotherapy. Rituximab is a monoclonal antibody that targets a protein called CD20. This protein is found on the surface of cancerous B-cells. The antibody works by recognizing and attaching to cells with CD20. The antibody then directs the immune system, to destroy the particular cell
• In some cases, stem cell transplants may be considered. This may be performed so that the individuals can undergo more intensive chemotherapy. In this form of treatment, very high doses of chemotherapy drugs are used, which results in a destruction of the bone marrow and stem cells along with the cancer cells. The individual is then usually given donor stem cells, or the individuals own stem cells, which were collected before the treatment
• Other drugs like rasburicase, antibiotics, and growth factors, may be used to address potential complications of chemotherapy

How can Burkitt Lymphoma be Prevented?

Currently, there are no specific methods or guidelines to prevent the formation of Burkitt Lymphoma.

What is the Prognosis of Burkitt Lymphoma? (Outcomes/Resolutions)

• The prognosis depends on the stage of the cancer, the individual’s health status, and their response to treatment
• About 60% of adults treated with intensive chemotherapy are cured of the lymphoma
• Children and adolescents are more likely to have better outcomes than adults, especially if the adults are over the age of 40 years
• The cure rate of Burkitt Lymphoma is almost 90% in developed countries, in comparison to a lower rate for other lesser developed regions
• Individuals with the cancer, which has involved sites outside of the lymphatic system, may have a poorer outcome
• If the lymphoma reappears after the treatment (comes back after initially responding to treatment), the prognosis is generally poor

Additional and Relevant Useful Information for Burkitt Lymphoma:

A recent clinical trial found that low-intensity chemotherapy is every effective and much less cytotoxic. However, since this only a recent finding and more research needs to be done for this type of treatment, the current standard of care is still intensive chemotherapy.