Types of Burkitt Lymphoma and How They Are Treated

Burkitt lymphoma (BL) is a rare but aggressive blood cancer most commonly seen in children. It is a form of non-Hodgkin lymphoma (NHL), a type of cancer that affects white blood cells known as lymphocytes. BL was first discovered in children in equatorial Africa and is closely associated with that region.

Burkitt lymphoma is closely associated with immune deficiency, such as among people with HIV or infectious mononucleosis. With the appropriate treatment, BL can be cured in up to 90% of cases. Adults are rarely affected but tend to have worse outcomes.

The article describes the types, symptoms, and causes of Burkitt lymphoma, including how this rare form of cancer is diagnosed and treated. It also looks at survival rates and ways to cope if you or your child is affected.

What Are the Types of Burkitt Lymphoma?

There are three main variants of Burkitt lymphoma—endemic, sporadic, and immunodeficiency—that differ in their location and the people they affect.

Endemic Burkitt Lymphoma

Endemic BL is the most common type that originated in Africa and is also widely seen in Papua New Guinea. It is rare outside either of those regions. Almost all cases occur in people with Epstein-Barr virus (EBV), the virus that causes infectious mononucleosis, or "mono."

The disease typically affects the jaw or other facial bones, but can also involve the abdomen, intestine, ovaries, kidneys, or breasts.

In equatorial Africa, around half of all childhood cancers are Burkitt lymphomas. The average age of diagnosis is 6 years.

Sporadic Burkitt’s Lymphoma

Sporadic BL is found all over the world. It mainly affects children and accounts for roughly a third of all lymphomas in children in the United States.

The association between sporadic BL and EBV is not as strong, with only 20% to 30% of cases positive for the virus. Males are affected three to four times more often than females. The median age of diagnosis is 10 years.

An abdominal tumor is a hallmark of sporadic BL. The bone marrow and meninges (the protective membrane surrounding the brain and spinal cord) are also sometimes affected.

Immunodeficiency Burkitt Lymphoma

Immunodeficiency BL is the type seen in people with advanced HIV infection or primary (inherited) immunodeficiency. It can also affect organ transplant recipients who take immunosuppressant drugs to avoid organ rejection.

Immunodeficiency BL tends to affect adults more than children, with most cases occurring between the ages of 40 and 45.

Immunodeficiency BL most often affects the gastrointestinal tract, lymph nodes, bone marrow, and central nervous system (comprised of the brain and spinal cord).

What Are the Signs and Symptoms of Burkitt Lymphoma?

Burkitt lymphoma is a fast-growing tumor, so symptoms tend to develop abruptly and progress rapidly. As a disease that primarily affects the lymphatic system, BL can cause swollen but painless lymph nodes in the head, neck, or other parts of the body.

Depending on which part of the body is affected, one or more large masses (tumors) can develop. With endemic BL, the mass will usually appear on the jaw, while swelling in the abdomen is characteristic of sporadic BL.

If the central nervous system is involved, the tumor can compress cranial nerves, causing involuntary movements of weaknesses known as palsies. This can cause an inability to blink or move your eyes in a certain direction or visual disturbances like double vision.

Other other symptoms of BL include:

• Fever
• Night sweats
• Unexplained weight loss
• Fatigue
• Shortness of breath

Causes and Risk Factors of Burkitt Lymphoma

As with NHL, no one is exactly sure why Burkitt lymphoma develops or why it affects certain populations more than others.

However, in recent years, genetic scientists have identified mutations of a gene, called the MSY gene, in between 70% and 90% of cases. This suggests that genetics plays a central role in the development of BL. This may account for why the disease is isolated to certain populations as the mutation is passed from one generation to the next.

Immune deficiency also appears to contribute given that BL is largely seen in people with compromised immune systems. It is thought that diseases like HIV can trigger BL in people with a genetic vulnerability to the disease, particularly young children with immature immune systems.

Malaria plays a role. The disease, widespread throughout Africa and Papua New Guinea, can reduce a person's resistance to EBV and cause more severe disease than what would be seen in places without malaria. This, in turn, could contribute to an increased risk of BL.

How Is Burkitt Lymphoma Diagnosed

If Burkitt lymphoma is suspected, the diagnosis will invariably involve a biopsy to obtain a tissue sample for evaluation in a pathology lab. The sample is usually obtained by inserting a hollow-core needle through the skin and into a tumor, lymph node, or bone marrow.

Under the microscope, the tissue sample will typically have a "starry sky" appearance with clusters of large white blood cells (called macrophages) filled with tiny, dead tumor cells.

Other tests may be ordered to characterize and stage the cancer, including:

• Computed tomography (CT) scan: A form of X-ray that can detect tumors in the chest, abdomen, and pelvis
• Positron emission tomography (PET) scan: An imaging study that can detect cancer based on increased blood circulation to growing tumors
  
• Spinal tap: A procedure used to extract cerebrospinal fluid (CSF) to check for cancer cells
• Lactic dehydrogenase (LDH) test: A blood test that would show increased levels of an enzyme called LDH if lymphoma is present
  

Staging Burkitt Lymphoma

Based on the findings from the pathology lab, the cancer specialist (called an oncologist) will stage the disease. The stage describes how advanced and widespread the cancer is and helps direct the appropriate course of treatment.

Burkitt lymphoma is broken down into four specific stages:

• Stage 1: Lymphoma cells are found in only one site in the body (usually the lymph nodes).
• Stage 2: Lymphoma cells are found in more than one site, but only on one side of the diaphragm.
• Stage 3: Lymphoma cells are found on both sides of the diaphragm.
• Stage 4: Lymphoma cells are found in the bone marrow or central nervous system.
  

How Is Burkitt Lymphoma Treated?

Burkitt lymphoma is highly treatable. However, because it grows so fast, immediate treatment is needed to avoid disease progression and potentially life-threatening complications like infection or organ failure.

Intensive chemotherapy is the first-line approach to treatment. Depending on your cancer stage, a combination of chemotherapy drugs would be prescribed in cycles, as follows:

• Stages 1 and 2: Two cycles of cyclophosphamide, vincristine, prednisolone, and doxorubicin
• Stages 3 and 4: Four cycles of cyclophosphamide, vincristine, prednisolone, doxorubicin, and high-dose methotrexate

Each cycle lasts for 2 to 4 weeks with a rest period in between.

Other drug combinations may be used. Some oncologists may incorporate a monoclonal antibody called Rituxan (rituximab) into the treatment plan to help stimulate the body's natural immune defense.

If there is no response to chemotherapy, a bone marrow transplant may be recommended.

Prognosis

The prognosis (outlook) for Burkitt lymphoma is generally good if treated promptly. In the developed world, children with early-stage disease have a survival rate of more than 90%. The survival rate for children with more advanced BL is still positive, hovering between 80% and 90%.

The outlook is less favorable for children in the developing world who lack access to the same treatments available in places like the United States. In low-income countries, the cure rate is closer to 50%.

Similarly, the prognosis is generally poor for adults with BL or people who experience a cancer relapse.

Coping and Living With Burkitt Lymphoma

Dealing with a cancer diagnosis can be difficult, but there are things you can do to better cope with treatment. This includes eating a healthy diet, exercising, and getting enough rest. Doing so will provide the energy needed to see you through chemotherapy. Caregivers should do the same.

You can also help reduce stress by practicing mind-body therapies like meditation or yoga, or by journaling your experience.

Summary

Burkitt lymphoma (BL) is a rare, aggressive form of non-Hodgkin lymphoma (NHL) that mainly affects children but can also affect adults. It often affects people with immune deficiency, such as those with HIV or infectious mononucleosis. BL is more common in equatorial Africa and Papua New Guinea, but certain types are found in the United States and other parts of the world.

BL can be diagnosed and staged with a biopsy and other tests. Treatment with chemotherapy is usually highly effective, offering cure rates of up to 90% in resource-rich countries.