This information is about a specific type of non-Hodgkin lymphoma (NHL) known as peripheral T-cell lymphoma. It should ideally be read with our general information about non-Hodgkin lymphoma.

Peripheral T-cell lymphoma accounts for about one in 100 of all cases of NHL. It can occur at any age from young adulthood to old age. It is slightly more common in men than in women.

It is a type of T-cell lymphoma, and the abnormal T-lymphocytes are found in the peripheral circulating blood. Peripheral T-cell lymphomas can also be subdivided into different types.

Some sub-types of peripheral T-cell lymphomas are quite rare in the western world. They are more common in the far east in countries such as Japan and China, where a viral infection called HTLV-1 is very common. HTLV-1 infection can make people more likely to develop some types of peripheral T-cell lymphoma.

Sometimes, T-cell lymphomas mainly affect the skin and are known as cutaneous T-cell lymphomas (CTCL).

The first signs of peripheral T-cell lymphoma can vary according to the type, but often the first sign of the condition is a painless swelling in the neck, armpit, or groin, caused by enlarged lymph nodes. Frequently, glands in several different areas are involved. The lymphoma commonly affects various organs in the body, such as the bone marrow, liver, spleen, stomach or bowel, and the skin. Some people experience a loss of appetite and tiredness.

Some people with this type of lymphoma will already have been diagnosed with other conditions affecting the immune system, or causing an overproduction of particular types of blood cells.

Other symptoms may include night sweats, unexplained high temperatures, and weight loss. These are described as B symptoms.

A diagnosis is made by removing an enlarged lymph node, or part of it, and examining the cells under a microscope (biopsy). 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, 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 affected.

• Stage 1 The lymphoma is only in one group of lymph nodes, in one particular area of the body.
• Stage 2 More than one group of lymph nodes is affected, but all the affected nodes are contained within either the upper half or the lower half of the body. The upper half of the body is above the sheet of muscle underneath the lungs (the diaphragm), and the lower half is below the diaphragm.
• Stage 3 Lymphoma is present in lymph nodes in both the upper and the lower parts of the body (ie in lymph nodes both above and below the diaphragm). The spleen is considered as a lymph node in this staging system.
• Stage 4 Lymphoma has spread beyond lymph nodes to other lymphatic organs, for example to sites such as the bone marrow, liver or lungs.

The stage usually includes the letter A or B, which describes whether the B symptoms are present or not (eg stage 2B). Sometimes the lymphoma can start in areas outside the lymph nodes, and this is represented by the letter E, which stands for extranodal (eg stage 3AE).

Grading

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

Peripheral T-cell lymphoma behaves as a high-grade lymphoma and usually needs prompt treatment.

Chemotherapy

Chemotherapy is the use of anti-cancer (cytotoxic) drugs to destroy cancer cells. It is the usual treatment for peripheral T-cell lymphoma. It can be quite difficult to treat successfully, although some people are cured by chemotherapy, and in others chemotherapy can often reduce and control the condition for a period of time.

A combination of chemotherapy drugs is usually used, such as CHOP or PMitCEBO. CHOP includes the drugs vincristine, doxorubicin, cyclophosphamide and prednisolone. PMitCEBO involves prednisolone, mitoxantrone, cyclophosphamide, etoposide, bleomycin and vincristine.

Radiotherapy

Radiotherapy is the use of high-energy rays to destroy cancer cells, while doing as little harm as possible to the healthy cells. If the lymphoma is stage 1 or 2 (only affecting lymph nodes in one area of the body), a combination of chemotherapy and radiotherapy may be used.

High-dose treatment with stem cell support

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

As side effects can be severe, this type of treatment is generally not given to people over the age of 60–65. This is because the intensity of the treatment increases the risks of serious side effects for people over this age.

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 peripheral T-cell 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 peripheral T-cell lymphoma, life may seem largely unaffected while, for others the diagnosis of cancer may be a cause of great fear and distress. If you would like to discuss the condition, its treatment, or the practical and emotional problems of living with peripheral T-cell 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.
• British Committee for Standards in Haematology Guidelines on Nodal Non-Hodgkin Lymphoma, Draft 2. August 2002.
• Improving Outcomes in Haemato-Oncology. National Institute of Clinical Excellence, November 2003.

For further references, please see the general bibliography.