Burkitt lymphoma is a type of blood cancer that falls under the category of non-Hodgkin lymphoma (NHL). It is an aggressive disease and may affect many different parts of the body. Burkitt lymphoma develops from B cells, which are immune cells that help the body fight off infection. People with Burkitt lymphoma produce too many B cells that don’t work as well as they should. These abnormal cells can crowd out other normal blood cells and fail to destroy germs like bacteria and viruses.
Burkitt lymphoma accounts for 1 percent to 5 percent of all cases of non-Hodgkin lymphoma. It is more common as a childhood cancer, making up 40 percent of all non-Hodgkin lymphoma seen in children and teens. Males are three to four times more likely than females to develop this disease.
There are three subtypes of Burkitt lymphoma: sporadic, endemic (or African), and immunodeficiency associated.
Sporadic Burkitt lymphoma affects people living in all parts of the world and is the most common type of Burkitt lymphoma seen in the United States and Western Europe in children. Young adults and children are more likely to get this subtype, although people of any age can be affected.
Endemic (or African) Burkitt lymphoma is the most common subtype. It is primarily seen in central Africa and is usually a pediatric disease, meaning it is more common in children.
The immunodeficiency-associated form is diagnosed in people who have conditions that impair the immune system. It most often develops in people with human immunodeficiency virus (HIV) infection and can also affect people who have undergone an organ transplant or have other immune disorders.
Another type of non-Hodgkin lymphoma is called non-Burkitt lymphoma or Burkitt-like lymphoma. It shares similarities with both Burkitt lymphoma and another blood cancer called diffuse large B-cell lymphoma (DLBCL). Burkitt-like lymphoma involves the same treatment as Burkitt lymphoma.
The different types of Burkitt lymphoma affect the body in various ways, leading to their own sets of symptoms. A common symptom is swollen lymph nodes. Lymph nodes are glands located throughout the body, in the neck, armpits, and groin. When they become swollen, they may look and feel like large lumps.
Children with sporadic Burkitt lymphoma usually develop a tumor in their abdomen. An abdominal tumor can block the intestines and cause these symptoms:
Adults with sporadic or immunodeficiency-associated Burkitt lymphoma also usually have fever, night sweats, and unexpected weight loss. In about one-third of people, the sporadic and immunodeficiency-related subtypes also affect the bone marrow (the tissue inside the bone that makes new blood cells). When the bone marrow is affected, there may be additional symptoms, including:
Endemic Burkitt lymphoma usually starts in the face or jaw. People with the endemic subtype may develop a large lump on their jawbone or another facial bone. This subtype may also lead to swelling around the eyes.
Symptoms of lymphoma vary from person to person. Some individuals may experience most or all of these symptoms, and others may not have any obvious symptoms. Some of these symptoms may also be caused by colds, the flu, or other types of blood cancer, so be sure to consult your doctor.
The first step toward a lymphoma diagnosis is a physical exam. Your doctor will likely ask you about your medical history, symptoms, and possible risk factors during this exam. Diagnosis of Burkitt lymphoma is made through a lymph node biopsy. A biopsy includes the removal of a small piece of the lymph node so that it can be examined under a microscope.
If your doctor finds lymphoma cells in your lymph nodes, they will probably do additional tests to determine your lymphoma grade (how quickly your cancer is growing) and stage (how far your cancer has already spread). Some of the tests that may be used to determine grade and stage include the following:
Tests such as a computed tomography (CT) scan or a positron emission tomography (PET) scan help doctors see where tumor cells are within your body.
A doctor may take a sample from the inside of your bones to see whether any cancer cells are in the marrow.
A lumbar puncture helps doctors see if there are cancer cells in your brain and spinal cord, together known as your central nervous system, or CNS.
Your doctor may use blood samples to determine whether you have abnormal levels of any blood cells and analyze whether your kidneys and liver are damaged.
Genetic testing helps your doctor learn more about what type of lymphoma you have.
All cancers are caused by gene mutations. In the case of Burkitt lymphoma, these gene mutations are not inherited, but occur throughout a person’s life. This means that Burkitt lymphoma rarely runs in families.
Approximately 95 percent of people with Burkitt lymphoma have changes in a gene called the c-MYC gene. These changes lead to cancer cell proliferation (fast growth). Other gene mutations have also been found in Burkitt lymphoma cancer cells, including changes in immunoglobulin genes, which help the immune system make infection-fighting antibodies.
Many of these gene changes are linked to infections from viruses. Sporadic Burkitt lymphoma often occurs in people who have previously been infected with Epstein-Barr virus (EBV), the virus that causes mononucleosis (also called mono). The endemic subtype is linked to infections with EBV or malaria. Immunodeficiency-related Burkitt lymphoma most often occurs in people who have HIV.
However, having a history of any of these infections does not necessarily mean you will develop Burkitt lymphoma. The majority of people with EBV, malaria, or HIV do not get lymphoma, and some people with no known risk factors will develop this disease. Most of the causes of lymphoma are outside anyone’s control.
Read more about the causes of Burkitt lymphoma.
Lymphomas all develop from special cells called lymphocytes. There are three main kinds of lymphocytes: B cells, T cells, and natural killer cells. Each type of lymphocyte plays a different role in the immune system, and each of these cells can become cancerous.
There are also two main types of lymphoma: Hodgkin lymphoma, which usually develops from B cells and non-Hodgkin lymphoma, which can develop from any type of lymphocyte. Within each of these two main categories, there are many subtypes of lymphoma classified based on several factors, including what type of lymphocyte is affected, how big the cancer cells are, what the cells look like, and how fast they grow. Burkitt lymphoma develops from B cells and leads to cancer cells that are small and grow quickly.
Although all lymphomas share some similarities, it is important to know which subtype of lymphoma you have because different types need different treatment plans and may have different outlooks.
Burkitt lymphoma is one of the fastest-growing types of cancer, so it’s important to start treatment right away. Many people with this disease are given intensive chemotherapy (anticancer drugs given at high doses). Some examples of these drugs include Cytoxan (cyclophosphamide), Oncovin (vincristine), VP-16 (etoposide), and Trexall (methotrexate). You may receive a combination of these or other chemotherapy medications.
Additionally, Burkitt lymphoma is often treated with a medication called Rituxan (rituximab). Rituxan is an antibody that can specifically target and destroy B cells, which are the cells that become cancerous in Burkitt lymphoma.
Clinical trials that are testing new therapies for Burkitt lymphoma may also be available.
There are various treatment goals for people with Burkitt lymphoma. In some cases, the goal may be to cure lymphoma, meaning that cancer cells are completely removed from the body. In other cases, treatment goals may include slowing down the cancer or controlling symptoms.
Read more about treatment options for Burkitt lymphoma.
Most people with Burkitt lymphoma who receive aggressive treatment can live for a long time. In particular, children with this disease have a good chance of survival following treatment. As many as 98 percent of children will survive if the disease is caught at an early stage (located in one part of the body, without spreading). Even if cancer has already spread, treatment can help 50 percent to 90 percent of children be disease-free for the long term.
Burkitt lymphoma is harder to treat in adults, and a person’s outlook typically depends on their age and stage.
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