Burkitt leukemia (sometimes called Burkitt’s leukemia or Burkitt lymphoma) is a rare type of leukemia that involves a type of white blood cells called B lymphocytes (B cells). Burkitt leukemia is very dangerous because of how aggressive it is compared to other types of leukemia and lymphoma. A diagnosis of Burkitt leukemia or lymphoma typically requires immediate treatment because it is a life-threatening medical emergency. Burkitt leukemia or lymphoma is one of the more common pediatric blood cancers.

What Are Burkitt Leukemia and Burkitt Lymphoma?

Burkitt leukemia is a subtype of acute lymphoblastic leukemia (ALL) that is also known as mature B-cell ALL. Burkitt leukemia is often associated with Burkitt lymphoma, a type of blood cancer that produces tumors. The names Burkitt leukemia and Burkitt lymphoma are often used interchangeably, although leukemia only involves the blood and bone marrow, and lymphomas form tumors. Both Burkitt leukemia and Burkitt lymphoma involve the same type of abnormal blood cells and are diagnosed and treated the same way. Burkitt leukemia and lymphoma are cancers caused by abnormal B lymphocytes, the type of white blood cells that produce antibodies. Burkitt leukemia and lymphoma can affect both children and adults at any age.

Burkitt Lymphoma

Burkitt lymphoma is a type of non-Hodgkin lymphoma (NHL) where a tumor forms from B lymphocytes, which can lead to Burkitt leukemia. These lymphomas can form throughout the body in lymphatic tissue (including lymph nodes, spleen, bone marrow, thymus, adenoids, tonsils, intestines, and stomach) and rapidly spread to other organs. Tumors in the abdomen occur in people throughout the world, whereas tumors of the jaw are most common in central Africa. Burkitt lymphoma is a fast-growing tumor that aggressively spreads to affect organs throughout the body.

There are three subtypes of Burkitt lymphoma: endemic, sporadic, and immunodeficiency-related.

• Endemic Burkitt lymphoma is found in Africa, usually in children. It is often associated with Epstein-Barr virus (EBV), a very common virus that can cause mononucleosis.
• Sporadic Burkitt lymphoma, or nonendemic Burkitt lymphoma, is very rare and occurs mostly in children. This type is sometimes associated with EBV.
• Immunodeficiency-associated Burkitt lymphoma occurs in people with compromised immune systems, such as people with human immunodeficiency virus (HIV), acquired immunodeficiency syndrome (AIDS), or people taking immunosuppressant drugs after an organ transplant. Burkitt lymphoma in people with HIV/AIDS is sometimes linked to EBV.

Symptoms

Symptoms of Burkitt leukemia and lymphoma include symptoms common to ALL and other types of leukemia, such as:

• Fatigue
• Dizziness or lightheadedness
• Shortness of breath
• Frequent or recurring infections
• Bone or joint pain
• Bruising easily (especially bruising without injury)
• New or unusual bleeding (nosebleeds, bleeding gums, heavy menstruation)

Other symptoms include:

• Swollen lymph nodes
• Enlarged spleen

Additional symptoms commonly seen with Burkitt lymphoma are:

• Abdominal pain
• Nausea
• Vomiting

Diagnosis

As with other types of leukemia and lymphoma, a diagnosis of Burkitt leukemia is made using several tests. These may include:

• Physical exam (especially feeling for lymph nodes and enlarged spleen)
• Blood tests, including complete blood count, blood smear, and blood chemistry
• Imaging, including X-rays, CT or CAT scan, and MRI
• Bone marrow biopsy (also called bone marrow aspiration)
• Spinal tap (also called lumbar puncture)
• Tumor biopsy, in cases of lymphoma
• Cytologic analysis (analysis of the cancer cells to determine the type of leukemia)

One of the most important steps in diagnosing any leukemia is identifying what type of cancer it is based on genetic markers and features unique to specific types of cancer cells.

Burkitt leukemia and lymphoma are identified by testing the cancer cells for specific genetic mutations of the c-Myc oncogene. These are changes in the DNA of the cancer cells that are the same for both Burkitt leukemia and lymphoma. Specific B-cell surface antigens such as CD19 and CD20, which are proteins on the outside of B cells, can also be used to distinguish Burkitt leukemia and lymphoma cells from other types of leukemias and lymphomas.

A correct diagnosis is important for ensuring the right treatment. For example, Burkitt leukemia or lymphoma is often difficult to distinguish from diffuse large B-cell lymphoma (DLBCL), but the conditions are treated differently.

Treatment and Outcomes

The primary treatment option for people with Burkitt leukemia is chemotherapy. There are many different chemotherapy regimens available that have a very high rate of successfully curing the disease. Generally, these are intensive chemotherapy treatments consisting of high-dose, short-duration treatments, including treatment of spinal fluid with chemotherapy (intrathecal chemotherapy).

Chemotherapy regimens used to treat Burkitt leukemia and lymphoma include:

• CODOX-M — Cyclophosphamide, vincristine, doxorubicin, and high-dose methotrexate
• CODOX-M/IVAC (Magrath protocol) — CODOX-M plus IVAC, ifosfamide, etoposide, and cytarabine
• HyperCVAD — Cyclophosphamide, vincristine, doxorubicin, dexamethasone, methotrexate, and cytarabine
• EPOCH — Etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin

In adults, antibody treatment with rituximab is often added to chemotherapy to improve effectiveness. Additionally, in the case of Burkitt lymphoma, surgery or radiation therapy may be used to reduce the size of the tumor.

In cases of relapsed Burkitt lymphoma, a stem cell transplant following high-dose chemotherapy may be appropriate.

Without treatment, Burkitt leukemia and lymphoma are fatal, but with treatment, survival rates are very high in adults and children.

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