Burkitt lymphoma

Burkitt lymphoma (BL) is a rare type of lymphoma that affects children and adults. It's more common in males.

BL develops when the body makes abnormal B-lymphocytes – a type of white blood cell that fights infection. The abnormal lymphocytes build up in one area of the body and form a lump (tumour). Other possible symptoms of BL include:

  • night sweats
  • high temperatures
  • weight loss
  • tiredness

To diagnose BL, a doctor removes a sample of tissue (biopsy). You will also have scans to find out how far the lymphoma has spread. This is called staging.

BL is a fast-growing (aggressive) lymphoma and needs to be treated quickly. Intensive chemotherapy is the main treatment. This is usually given as an inpatient. A monoclonal antibody drug called rituximab (Mabthera ®) may also be given. Some people may have a stem cell transplant. But this is an intensive treatment and not appropriate for everyone.

If a new treatment for BL is being tested, your doctor may invite you to take part in a clinical trial.

Burkitt lymphoma and Burkitt’s-like lymphoma

Burkitt lymphoma (BL) and Burkitt's-like lymphoma are rare types of non-Hodgkin lymphoma. This information should ideally be read with our general information about non-Hodgkin lymphoma.

We hope this information answers your questions. If you have any further questions, you can ask your doctor or nurse at the hospital where you are having treatment.


Non-Hodgkin lymphoma

Non-Hodgkin lymphoma is a cancer of the lymphatic system. This is part of the body’s immune system, which helps us fight infection. The lymphatic system is made up of organs such as the bone marrow, thymus, spleen and lymph nodes (sometimes called lymph glands).

The lymphatic system
The lymphatic system

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The lymphatic system

There are lymph nodes all over the body. They are connected by a network of tiny tubes (lymphatic vessels) that contain lymph fluid. As lymph fluid flows through the lymph nodes, the nodes collect and filter out things that the body doesn't need. These include bacteria, viruses, damaged cells and cancer cells.

Lymph fluid contains lymphocytes. These are white blood cells that help the body fight infection and disease. Lymphocytes grow in the bone marrow, where blood cells are made. The two main types of lymphocytes are B-cells and T-cells. B-cells mature in the bone marrow, and T-cells mature in the thymus gland behind the breastbone. When they're mature, both B-cells and T-cells help fight infections.

Lymphoma is a disease in which either T-cells or B-cells grow in an uncontrolled way.

There are many different types of non-Hodgkin lymphoma. They're grouped (or classified) according to features such as the type of cell involved (B-cells or T-cells) and how quickly the lymphoma grows.


Types of Burkitt lymphoma

Burkitt lymphoma (BL) is a rare type of non-Hodgkin lymphoma that affects the B-cells. It affects both children and adults. It's more common in men than in women.

There are three different types of Burkitt lymphoma.

Endemic BL

This is found in central Africa, usually in children, and is strongly linked to reduced resistance to a common virus called the Epstein-Barr virus (EBV). The jaw bone is often affected, which is rare in other types of BL.

Epstein-Barr is a common virus that causes glandular fever. It is not known why it increases the risk of lymphoma in some people but not others.

Sporadic BL

This is also linked with the Epstein-Barr virus but less clearly than endemic BL. This type of BL is more common in the UK.

Immunodeficiency-associated BL

This type of BL may occur in people who have had an organ transplant and are taking medicines to stop their immune system rejecting the transplant. It may also occur in people who have reduced immunity because they have HIV or AIDS.

We have separate information about HIV-related lymphomas, as they may need different treatment.


Signs and symptoms of Burkitt lymphoma

BL causes different symptoms depending on where in the body it is. The most common early symptom is swelling in the lymph nodes.

If BL is in lymph nodes in the chest, throat or jaw, this may cause a sore throat, difficulty swallowing or breathlessness.

The lymphoma often appears as a lump or mass in the tummy area (abdomen). This can cause pain and swelling, feeling sick (nausea) and diarrhoea.

Some people may also have night sweats, high temperatures or weight loss. These are known as B symptoms.


How Burkitt lymphoma is diagnosed

The most common test to diagnose Burkitt lymphoma is a biopsy. Your doctor will remove part or all of an enlarged lymph node or some tissue from a lump (tumour). This involves a small operation, which may be done under local or general anaesthetic. The lymph node or tissue is then examined under a microscope. Biopsies may also be taken from other areas of the body.

You may have some other tests to find out more about the type of lymphoma and how far it has spread. These may include blood tests, x-rays, scans, a lumbar puncture and bone marrow biopsy.

A lumbar puncture checks for lymphoma cells in your brain and spinal cord. The spinal cord and brain are surrounded by a fluid called cerebrospinal fluid (CSF). Your doctor uses a local anaesthetic to numb the lower part of your back and then passes a needle gently into your spine. They then take a small sample of CSF to be checked for lymphoma cells.

Your doctor may want to take a sample of bone marrow to examine under a microscope. The sample is usually taken from the back of your hip bone (pelvis). You will be given an injection of local anaesthetic to numb the area. The doctor will then pass a needle through the skin into the bone, and draw a small sample of liquid marrow (bone marrow aspirate) into a syringe.

After this, the doctor will also take a small core (piece) of marrow from the bone (a trephine biopsy). Both samples will be looked at later under a microscope.

Bone marrow sampling
Bone marrow sampling

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A sample of bone marrow is usually taken from the back of the hip bone.


Staging and grading of Burkitt lymphoma

Staging

Your doctors will look at how many groups of lymph nodes are affected, and if the lymphoma is in other organs. They also look at whether the affected lymph nodes are above or below the diaphragm (the sheet of muscle under the lungs). This is called staging. The staging system that's usually used for BL is called the Ann Arbor staging system. Different staging systems may be used for children and teenagers.

A ‘group of lymph nodes’ refers to lymph nodes in one area of the body such as in the armpit, on one side of the neck or in the groin. Burkitt lymphoma can start in areas outside the lymph nodes. Doctors call these extranodal sites.

Stage 1

One group of lymph nodes is affected.

Stage 2

Two or more groups of lymph nodes are affected on the same side of the diaphragm..

Stage 3

Lymph nodes above and below the diaphragm are affected.

Stage 4

The lymphoma has spread outside the lymph nodes to other areas such as the liver or lungs, or the brain or spinal cord (the central nervous system).

Grading

Doctors also divide non-Hodgkin lymphomas into one of two groups, depending on how quickly they grow:

  • indolent (low-grade), which are usually slow-growing
  • aggressive (high-grade), which grow quickly.
  • Burkitt lymphoma is an aggressive lymphoma that needs to be treated quickly.


Treatment for Burkitt lymphoma

Chemotherapy is the main treatment for BL. Usually an intensive treatment, which means staying in hospital for a few weeks, will be given. A monoclonal antibody drug called rituximab (Mabthera ®) may be given in addition to chemotherapy. Some people may have stem cell treatment.

Chemotherapy is the use of anti-cancer drugs to destroy cancer cells. The combination of drugs you have will depend on the stage of the lymphoma and how well you are to cope with the side effects. The chemotherapy drugs are given into a vein (intravenously). The following drugs may be used in different combinations to treat BL:

  • cyclophosphamide
  • vincristine
  • doxorubicin
  • methotrexate
  • ifosfamide
  • cytarabine
  • etoposide.

Your specialist will talk to you about which combination of the drugs you will have.

Chemotherapy can also be given into the spinal fluid to allow the drug to reach the spinal cord and brain (central nervous system). This is called intrathecal chemotherapy. It is done either to prevent lymphoma cells from spreading into the cerebrospinal fluid or to treat it if it already has.

Monoclonal antibody therapy

Monoclonal antibodies such as rituximab (Mabthera®) may target certain proteins on the surface of cancer cells. They can either make chemotherapy work better or stimulate the body’s immune system to destroy the cancer cells.

Steroid therapy

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

Tumour lysis syndrome (TLS)

Chemotherapy may cause the cancer cells to break down very quickly. This can cause chemical imbalances in the blood that affect the kidneys and the heart. This is called tumour lysis syndrome (TLS).

To reduce the risk of TLS, your doctor may give you a drug called rasburicase as a drip (infusion). You will also be given fluids through your drip to help protect your kidneys. You may only need rasburicase with the first treatment. After that, you can have tablets called allopurinol (Zyloric®) instead.

Stem cell treatment (transplants)

Some people with lymphoma may have treatment using stem cells. Stem cells are blood cells at their earliest stage of development. All blood cells develop from stem cells.

There are two different types of stem cell treatment, but they are not suitable for everyone.

Using your own stem cells

Some people have some of their own stem cells collected and stored. This allows them to have higher doses of chemotherapy to destroy the lymphoma cells. After the chemotherapy, their stem cells are returned by a drip (like a blood transfusion) to help their blood cells recover from the effects of chemotherapy. This is called high-dose chemotherapy with stem cell support.

Using stem cells from a donor

Some people may have treatment using stem cells from another person (a donor). This is called a donor (allogeneic) stem cell transplant.


Clinical trials for Burkitt lymphoma

New treatments for Burkitt lymphoma are being researched all the time. Your doctor may invite you to take part in a clinical trial to compare a new treatment against the best available standard treatment.


Information and support

Everyone has their own way of dealing with their illness and the different emotions they experience. You may find it helpful to talk things over with family and friends or your doctor or nurse. You can also contact our cancer support specialists or the organisations listed below for more information and support.


Useful organisations

The Lymphoma Association

The Lymphoma Association gives emotional support, advice and information on all aspects of Hodgkin lymphoma and non-Hodgkin lymphoma. It has a national network of people with lymphoma and local groups.

Leukaemia CARE

Leukaemia CARE is a national group promoting the welfare of people with leukaemia and other blood disorders, including non-Hodgkin lymphoma. Has regional support groups in many counties.