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Burkitt lymphoma (BL) is an uncommon form of non-Hodgkin lymphoma (NHL). It is a malignant con-dition (cancer) affecting a type of white blood cells called lymphocytes. This information is about BL in adults, unless otherwise stated.
The lymphatic system is a network of fine vessels, glands and channels which occur throughout the body. It also conveys nutrients and cells, and is responsible for draining fluid and waste products away from tissues, and into the blood stream to be processed. The lymphatic system is an important part of the immune system and is made up of lymph nodes and vessels and of collections of lymphocytes in other tissues.
Burkitt lymphoma is a high-grade form of NHL, this means that it develops quickly and, if not treated, it grows quickly. It is named after a medical missionary called Denis Burkitt, who first described the disease in the 1950s. Males are more likely to develop BL. Burkitt lymphoma often occurs in places other than lymph nodes, such as lymphatic tissue in the gut and the bone marrow.
There are three types of BL:
Unless otherwise stated, this information relates to sporadic BL and immunodeficiency-associated BL.
Virtually all cases of endemic BL are thought to be caused by EBV infection, probably with other factors such as malaria also being involved. Although some cases of sporadic or immunodeficiency-associated BL may be caused by EBV, the link is much less strong than for endemic BL. Non-endemic BL may be linked to other virus infections, although this has not been shown.
Immunodeficiency-associated BL may be caused by any condition which weakens the immune system, but it is most commonly seen as part of AIDS.
It is important to understand that:
The most common symptom of endemic Burkitt lymphoma is a swelling in the jaw, around the eyes, or in the abdomen (belly). In sporadic BL the most commonly affected site is the gut, which leads to abdominal pain, and sometimes nausea and vomiting or bleeding.
Other symptoms may include:
The most important test for diagnosis of BL is a biopsy of affected tissue. This may mean removing a whole abnormal lymph node (excision biopsy) or just a sample of tissue.
Other tests which may be done include:
Staging is the use of test results to show how widely the lymphoma has spread. There are four stages in the standard system used for adult Burkitt lymphoma:
Stages 1-2 are known as early-stage disease and stages 3-4 are known as advanced-stage disease.
This stage description may be modified by adding the letters A, B, E or S:
A modification of this system, called the St Jude system, is used to describe the stages for children with Burkitt lymphoma.
Burkitt lymphoma is a very aggressive (fast-growing) form of NHL but fortunately, it usually responds well to chemotherapy and is usually curable. The main ways in which BL is treated are:
Chemotherapy is the use of cell-killing drugs. These kill the cancer cells and/or stop them from dividing. Chemotherapy is usually given in blocks or ‘cycles’ of treatment. One cycle of treatment will consist of a series of doses of chemotherapy followed by a break for the healthy cells to recover. For patients who are well enough, chemotherapy for BL uses high-doses of drugs, which will require admission to hospital and careful daily monitoring. Older patients, or those who are less fit, may be treated with lower doses – although still effective for most patients, the cure rate is lower than for high-dose treatment.
Chemotherapy is normally given as a combination of drugs, which will usually include steroids. Steroids used to treat lymphoma are a laboratory-made version of chemicals naturally made by the body. They are very different from the type of steroids sometimes misused by body-builders or sportsmen. Because of the highly aggressive nature of the disease chemotherapy can cause a syndrome called tumor lysis syndrome which can affect the kidneys and therefore before initiation of steroids /chemotherapy a drug called rasburicase should be given to prevent tumor lysis
Patients who have BL and AIDS can normally have high-dose chemotherapy as long as they are being treated at the same time for their AIDS.
Stem cell transplant is mainly used in treatment of relapse of BL.
The details of your treatment will vary depending on whether your BL is sporadic or immunodeficiency-associated and on your general fitness. You will be given a chance to discuss treatment options and detailed information on your treatment plan before it starts. The side effects of treatment vary between different types of treatment and different patients. You will be given detailed information about any likely side effects before you start treatment.
We understand going through a blood cancer through journey can be difficult. It may help to talk to a close friend or relative about how you are feeling. Here are some questions that may be useful to ask your doctor.