This information is about specific types of non-Hodgkin lymphoma known as Burkitt lymphoma and Burkitt-like lymphoma. It should ideally be read with our general information about non-Hodgkin lymphoma (NHL).

Burkitt lymphoma is a type of B-cell lymphoma.

In 1956, a British surgeon called Dennis Burkitt was working in central Africa. He described an unusual type of lymphoma, which was very common in children. This became known as Burkitt lymphoma.

Later research showed that B-lymphocytes in these children had been infected with the Epstein-Barr virus (or E-B virus). Epstein-Barr virus infections are known as glandular fever or infectious mononucleosis. They are common and usually cause no long-term problems, but in central Africa many of the children had chronic malaria infections, which reduced their resistance to the virus. In some cases, this allowed the virus to change the infected B-lymphocytes into cancerous cells, leading to the development of the lymphoma. This is known as classical African or endemic Burkitt lymphoma.

In one type of non-Hodgkin lymphoma, the tumour cells have very similar appearances under the microscope to those of classical African or endemic Burkitt lymphoma. This rare condition is still called Burkitt lymphoma but is known as the non-African, or sporadic, type.

It seems that in this condition, once again, the Epstein-Barr virus infection can develop because the patient has reduced immunity. The Epstein-Barr virus is able to survive and ‘transforms’ the normal B-lymphocytes into cancerous cells. However, in the Burkitt’s lymphoma seen in the UK, the way normal B-lymphocytes change to cancer cells is less clear, and not all cases occur in people who have been in contact with the Epstein-Barr virus.

In the Western world, the most common symptom is an abdominal swelling that starts in the bowel. Burkitt lymphoma may also affect other organs such as the eye, the ovaries, kidneys, and glandular tissue such as the breast, thyroid or tonsil. The classical African or endemic type of Burkitt lymphoma usually affects the jawbone. It can spread to the nervous system, damaging the nerves and causing possible weakness or paralysis. It may also affect the lymph nodes or bone marrow. Some people experience a loss of appetite and tiredness.

Other symptoms, which are known as B symptoms, include sweating at night, unexplained high temperatures, and weight loss.

A diagnosis is made by removing an enlarged lymph node, or part of it, and examining the cells under a microscope (biopsy). You will be referred to a surgeon for this procedure. It is a very small operation and may be done under local or general anaesthetic. Biopsies may also be taken from other body tissues.

Additional tests, including blood tests, x-rays, scans, lumbar punctures, and bone marrow samples, are then used to get more information about the type of lymphoma and how far it has spread in the body. This information is used to help decide which treatment is most appropriate for you.

Staging

The stage of NHL describes how many groups of lymph nodes are affected, where they are in the body, and whether other organs such as the bone marrow or liver are involved. The staging system described here is known as the 'St Jude Modification of Ann Arbor' staging system and is widely used for this type of lymphoma; particularly in children and young people.

Sometimes the lymphoma can start in areas outside the lymph nodes and this is known as extranodal disease. Nodal refers to lymphoma affecting the lymph nodes.

Stage 1 There is a single extranodal tumour or there is a nodal lymphoma in one area of the body (but not including the central lymph nodes within the chest – the mediastinum – or the abdomen).

Stage 2 Can be any of the following:

• There is a single extranodal tumour and nearby lymph nodes are affected.
• There are two single extranodal tumours on the same side of the diaphragm (with or without nearby lymph nodes being affected).
• The lymphoma started in the stomach or intestines – nearby nodes may or may not be affected.
• The lymphoma is in two or more areas of lymph nodes on the same side of the diaphragm.

Stage 2R The lymphoma was in the abdominal area but has been completely removed by surgery.

Stage 3 Can be any of the following:

• There are two single extranodal tumours on opposite sides of the diaphragm.
• The lymphoma started in the lungs, chest area or thymus gland.
• The lymphoma is affecting the area within or around the spinal cord.
• The lymphoma started in the abdomen and affects a large area.
• Two or more nodal areas are affected on opposite sides of the diaphragm.

Stage 3A The lymphoma is in the abdominal area only and cannot be removed by surgery.

Stage 3B The lymphoma is affecting several organs within the abdomen.

Stage 4 Any of the above, plus – at the time of diagnosis – the central nervous system (brain and spinal cord) and/or the bone marrow are also affected.

Grading

For practical purposes, non-Hodgkin lymphomas are divided into two groups: low- and high-grade. Low-grade lymphomas are usually slow-growing, and high-grade lymphomas grow more quickly.

Burkitt lymphoma is a high-grade lymphoma. This means that it is faster-growing and usually needs prompt treatment with chemotherapy.

Chemotherapy

Chemotherapy is the use of anti-cancer (cytotoxic) drugs to destroy cancer cells. It is the main treatment for this type of lymphoma. The chemotherapy treatment may differ slightly depending on how many areas of the body are affected. It is usually quite intensive and is likely to involve a stay in hospital. Usually the chemotherapy drugs are given into a vein (intravenously). As there is a risk that this type of lymphoma may spread into the nervous system, chemotherapy drugs are also usually injected into the fluid around the spinal cord. This is known as intrathecal chemotherapy.

The following drugs may be used in various combinations to treat Burkitt lymphoma: cyclophosphamide, vincristine, doxorubicin, methotrexate, cytarabine, ifosfamide and etoposide.

Monoclonal antibody therapy

Another treatment that is often used is a monoclonal antibody called rituximab (Mabthera®). Monoclonal antibodies are drugs that recognise, target, and stick to specific proteins on the surface of cancer cells, and can stimulate the body’s immune system to destroy the cell. This treatment is usually given with chemotherapy.

High-dose treatment with stem cell support

High-dose chemotherapy with bone marrow or stem cell infusions has been used for some patients. This type of treatment involves very intensive chemotherapy, and sometimes radiotherapy.

As the side effects can be severe, some types of high-dose treatment are not given to people over the age of 45–50 while others can be given to people of up to 65 who are fit enough. The intensity of the treatment increases the risks of serious side effects for people over these ages.

Steroid therapy

Steroids are drugs which are often given with chemotherapy to help treat lymphomas. They also help you to feel better and can reduce feelings of sickness.

New treatments for Burkitt lymphoma are being researched all the time, and you might be invited by your doctor to take part in a clinical trial to compare a new treatment against the best available standard treatment. Your doctor must discuss the treatment with you, and have your informed consent before entering you into any clinical trial.

The need for practical and emotional support will of course be individual. For some people with Burkitt lymphoma, life may seem largely unaffected; for others the diagnosis of cancer may be a cause of great fear and distress. If you would like to discuss your condition, its treatment, or the practical and emotional problems of living with Burkitt lymphoma, please contact our cancer support service.

This section has been compiled using information from a number of reliable sources, including:

• Oxford Textbook of Oncology (2nd edition). Eds Souhami et al. Oxford University Press, 2002.
• Wintrobe’s Clinical Haematology (11th edition). Eds Lee et al. Williams and Wilkins, 2004.
• Improving Outcomes in Haemato-Oncology. National Institute of Clinical Excellence, November 2002.

For further references, please see the general bibliography.