Burkitt lymphoma is a type of mature B-cell non-Hodgkin lymphoma (NHL). Burkitt lymphoma develops most often in children or young adults and is uncommon in older adults. It occurs most often in boys. Epstein-Barr virus (EBV) is a known risk factor, and most people diagnosed with Burkitt lymphoma are infected with this virus.
Burkitt lymphoma is a very fast-growing (aggressive) type of NHL. It tends to occur in organs or tissues other than the lymph nodes (called extranodal sites). It often spreads to the brain or spinal cord (called the central nervous system, or CNS).
Burkitt lymphoma can affect the bone marrow so that it doesn’t work normally. As a result, blast cells don’t mature properly, and these abnormal immature blood cells (called Burkitt lymphoma cells) are found in the circulating blood. If more than 25% of the cells in the bone marrow are Burkitt lymphoma cells, doctors may classify it as acute lymphocytic leukemia (ALL), L3 or Burkitt-type leukemia.
There is also a Burkitt-like lymphoma. When viewed under a microscope, Burkitt-like lymphoma cells look somewhat different from Burkitt lymphoma cells. They also have some features of diffuse large B-cell lymphoma (DLBCL) cells. Burkitt-like lymphoma is grouped with Burkitt lymphoma.
Subtypes of Burkitt lymphoma
There are 3 subtypes of Burkitt lymphoma.
Endemic Burkitt lymphoma is also called African Burkitt lymphoma because it mainly occurs in Africa. It is almost always linked with Epstein-Barr virus infection. It generally starts as a tumour on the jawbone or another bone in the face.
Sporadic Burkitt lymphoma is also called non-African, or non-endemic, Burkitt lymphoma. It is the most common type of Burkitt lymphoma in North American and European countries. It is sometimes linked with the Epstein-Barr virus, but many people who develop sporadic Burkitt lymphoma are not infected with this virus. This type of NHL most often starts as a tumour in the abdomen, but it can also start in the ovaries, testicles, kidneys or other organs. It can spread to the CNS.
Immunodeficiency-associated Burkitt lymphoma tends to occur in people with weakened immune systems, typically those with HIV infection. It is not usually linked to the Epstein-Barr virus. The number of cases of immunodeficiency-associated Burkitt lymphoma seems to be dropping because antiretroviral therapy is used to treat HIV.
Chemotherapy is the main treatment for Burkitt lymphoma. Targeted therapy and CNS prophylaxis may also be given.
Combinations of chemotherapy drugs that may be used to treat Burkitt lymphoma include:
- CODOX-M – cyclophosphamide (Cytoxan, Procytox), vincristine (Oncovin), doxorubicin (Adriamycin) and high-dose methotrexate
- CODOX-M/IVAC (Magrath protocol) – CODOX-M plus IVAC, which is ifosfamide (Ifex), etoposide (Vepesid) and cytarabine (Cytosar, Ara-C)
- hyperCVAD – cyclophosphamide, vincristine, doxorubicin, dexamethasone (Decadron, Dexasone), methotrexate and cytarabine
- EPOCH – etoposide, prednisone, vincristine, cyclophosphamide and doxorubicin
Tumour lysis syndrome (TLS) is a group of side effects that develops when a large number of lymphoma cells are killed in a short period of time. It often develops with chemotherapy for Burkitt lymphoma, so the healthcare team will take measures to prevent TLS before chemotherapy starts. Find out more about tumour lysis syndrome.
Targeted therapy uses drugs to target specific molecules (such as proteins) on the surface of cancer cells. These molecules help send signals that tell cells to grow or divide. By targeting these molecules, the drugs stop the growth and spread of cancer cells while limiting harm to normal cells.
Rituximab (Rituxan) is the most common targeted therapy used to treat Burkitt lymphoma. It may be added to any of the above combinations of chemotherapy.
Central nervous system prophylaxis
CNS prophylaxis is used to try to prevent the lymphoma cells from entering the tissue covering the brain and spinal cord.
Methotrexate and cytarabine are used for CNS prophylaxis. They are given as intrathecal chemotherapy. This means that they are injected into the cerebrospinal fluid (CSF).
High-dose methotrexate is also used for CNS prophylaxis. It is given by a needle in a vein (intravenously).
A type of herpes virus that causes mononucleosis (a highly infectious disease that causes fever, fatigue, malaise and sore throat).
Epstein-Barr virus is associated with an increased risk of certain cancers, including Burkitt lymphoma.
Also called Human herpesvirus 4 (HHV-4).
An immature bloodcell.
Different types of blast cells develop into different types of blood cells. For example, lymphoblasts develop into lymphocytes and erythroblasts develop into erythrocytes.
Also called a blast.