Burkitt lymphoma: what it is, symptoms, diagnosis and treatment

Lymphoma is a cancer of the lymphatic system, which is part of the immune system. Cancer Research UK explains that non-Hodgkin lymphoma (NHL) develops when white blood cells called lymphocytes grow out of control. Burkitt lymphoma arises from abnormal B lymphocytes, so it is a type of B-cell lymphoma, and the abnormal cells build up in lymph nodes or other organs and do not fight infection normally.

Doctors classify NHL as low grade (slow growing) or high grade (fast growing). Burkitt lymphoma is high grade. That is why it is treated as soon as it is diagnosed, and why the treatment is intensive.

Cancer Research UK and Blood Cancer UK both describe three main types. They share many symptoms and treatments but have different causes.

• Sporadic: the most common type in the UK. It is sometimes linked to the Epstein-Barr virus (EBV), the virus that causes glandular fever, though EBV is very common and most people who have it never develop lymphoma.

• Immunodeficiency-related: develops in people whose immune system is not working as well as it should, for example with HIV or after an organ transplant.

• Endemic: most common in children in parts of Africa, with a possible link to malaria and EBV, and rare in the UK.

Cancer Research UK says sporadic Burkitt lymphoma often grows in the abdomen and bowel. Blood Cancer UK says symptoms mainly depend on where the lymphoma cells collect, and usually develop quickly.

• Tummy or back pain, feeling or being sick, diarrhoea, swelling of the tummy and bleeding.

• Painless swellings in the neck, armpit or groin, which are enlarged lymph nodes and can grow very quickly.

• B symptoms: heavy night sweats, fevers that come and go with no obvious cause, and losing a lot of weight without trying.

• Tiredness, breathlessness, bruising or bleeding if lymphoma cells in the bone marrow reduce normal blood cell counts.

The main test is a biopsy, in which a doctor removes a sample from the affected area, often part or all of a swollen lymph node, for a specialist to examine under a microscope. Blood Cancer UK says it is done under local anaesthetic in most cases, and is the only way to confirm Burkitt lymphoma.

If the diagnosis is made, you usually have further tests to find out where the lymphoma is, which is called staging. Cancer Research UK lists a PET-CT scan, a bone marrow test, an MRI of the head or spine and a lumbar puncture to check for lymphoma cells in the fluid around the brain and spinal cord. There are four main stages, and doctors also consider whether the lymphoma is low risk or high risk using factors such as performance status, the blood level of an enzyme called LDH, whether it is outside the lymph nodes and the stage.

Because Burkitt lymphoma grows fast, treatment starts soon after diagnosis. Cancer Research UK says the main treatments are chemotherapy combined with steroids and a targeted cancer drug, called chemo-immunotherapy, and chemotherapy to stop the lymphoma spreading to the brain and spinal cord. Common regimens include R-CODOX-M with or without R-IVAC, and DA-EPOCH-R, all of which include the targeted drug rituximab.

Blood Cancer UK says that for low-risk disease, or if intensive chemotherapy cannot be tolerated, a less intensive regimen called R-CHOP may be used. Treatment is intensive and may last several months, often with several weeks in hospital. Your team selects the regimen based on your stage, risk group, fitness and preferences.

If the lymphoma does not go away or comes back, Cancer Research UK says further treatment may include different chemo-immunotherapy combinations or a stem cell transplant.

Blood Cancer UK explains that tumour lysis syndrome can happen when treatment, or the lymphoma itself, causes a sudden change in the balance of chemicals in the blood. Chemotherapy can kill many lymphoma cells quickly, and the chemicals they release can strain the kidneys and heart. If your risk is high you have regular blood tests, and your team can give medicines to prevent or treat it.

Blood Cancer UK says that for many people treatment can get rid of the cancer cells, but that the outlook is very individual and depends on age, personal circumstances and stage. The hospital team is the best source of an estimate for your own situation.

The treatment can affect fertility and cause side effects such as sickness, hair loss, a higher risk of infection and fatigue; Blood Cancer UK suggests asking about fertility options before treatment starts if you wish to have children. Support is available from nurses and charities throughout treatment and afterwards.

Burkitt lymphoma is treated by specialist haematology teams. Cancer Research UK says that if you have Burkitt lymphoma you have treatment soon after diagnosis, and Blood Cancer UK says that most people will need intensive treatment at the time of diagnosis.

Private care is sometimes used for a second opinion, but whichever route is taken, the treatment plan is made with a specialist multidisciplinary team. Your hospital team is the best source of information on your own outlook.