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This information is about a specific type of non-Hodgkin lymphoma (NHL), known as mantle cell lymphoma. It should ideally be read with our general information about non-Hodgkin lymphoma.
Mantle cell is an rare type and accounts for about one in 20 of all cases of non-Hodgkin lymphomas. It is a cancer of the B-lymphocytes. Mantle cell lymphoma can occur at any age from the late 30s to old age, but is more common in the over 50s. It is three times more common in men than in women.
The causes of mantle cell lymphoma are unknown. Mantle cell lymphoma, like other cancers, is not infectious and cannot be passed on to other people.
The first sign of the condition is often a painless swelling in the neck, armpit or groin, caused by enlarged lymph nodes. Often more than one group of nodes is affected. The lymphoma may spread to affect various organs in the body, such as the bone marrow, liver or spleen. About 1 in 4 people with mantle cell lymphoma will also have the disease in the stomach or bowel. Some people experience a loss of appetite and tiredness.
Other symptoms may include night sweats, unexplained high temperatures, and weight loss. These are known as B symptoms.
A diagnosis is made by removing an enlarged lymph node, or part of it, and examining the cells under the 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.
The stage of non-Hodgkin lymphoma 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 stage usually includes the letter A or B, which describes whether or not any B symptoms are present (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).
For practical purposes non-Hodgkin lymphomas are also divided into one of two groups: low- and high-grade. Low-grade lymphomas are usually slow-growing, and high-grade lymphomas tend to grow more quickly.
Mantle cell lymphoma is a low-grade lymphoma, but may behave in a more aggressive way, like a faster-growing lymphoma.
Chemotherapy is the use of anti-cancer (cytotoxic) drugs to destroy cancer cells. It is the most commonly used treatment for mantle cell lymphoma. The type of chemotherapy depends upon the extent of the lymphoma, and other factors, such as your age and general health. Usually, quite an intensive form of chemotherapy is needed.
There are a number of different combinations of chemotherapy that may be given. A commonly-used combination is the R-CHOP regimen. This includes the chemotherapy drugs vincristine, doxorubicin, cyclophosphamide, the steroid prednisolone, and the monoclonal antibody rituximab (Mabthera®).
A chemotherapy drug called fludarabine (Fludara®) is sometimes used. It may be given in combination with cyclophosphamide and rituximab. This is sometimes known as the FCR regime. Another combination of drugs that may sometimes be used, usually in younger people, is the Hyper-CVAD regimen. It can be used on its own or before a stem cell transplant (see below).
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 years who are fit enough. This is because the intensity of the treatment increases the risks of serious side effects for people over this age.
Radiotherapy is the use of high-energy rays to destroy cancer cells, while doing as little harm as possible to the healthy cells. It may be used when the lymphoma cells are contained in one or two groups of lymph nodes in the same part of the body (stage 1 or 2). It may also be given in addition to chemotherapy.
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.
Another treatment that may be used is a monoclonal antibody called rituximab (Mabthera®). Monoclonal antibodies are drugs that recognise, target, and stick to particular 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 as part of the R-CHOP regimen.
Interferon is a protein that occurs naturally in the body. It is sometimes used to boost the body’s own immune system to control the lymphoma. Interferon is given as an injection just under the skin (subcutaneously).
New treatments for mantle 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 mantle cell 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 the condition, its treatment, or the practical and emotional problems of living with mantle cell lymphoma, please contact our cancer support service.
This section has been compiled using information from a number of reliable sources, including:
For further references, please see the general bibliography.
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