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This information is about a specific type of non-Hodgkin lymphoma (NHL), known as nodal marginal zone B-cell lymphoma. It should ideally be read with our general information about non-Hodgkin lymphoma.
Nodal marginal zone B-cell lymphoma is very rare, accounting for about 1 in 100 of all cases of non-Hodgkin lymphoma. It used to be called monocytoid B-cell lymphoma and is a cancer of the B-lymphocytes. It can occur in middle age or in older people. It is slightly more common in women than in men.
Lymphoma is a disease in which either T- or B-lymphocytes grow in an uncontrolled way and cause various parts of the lymphatic system to enlarge or stop working effectively.
There are more than 20 different types of non-Hodgkin lymphoma. Nodal marginal zone B-cell lymphoma is very rare, making up about 1% of all cases of non-Hodgkin lymphoma. It used to be called monocytoid B-cell lymphoma and is a cancer of the B-lymphocytes. It can occur in middle age or in older people. It is slightly more common in women than men.
The causes of nodal marginal zone B-cell lymphoma are unknown. Nodal marginal zone B-cell lymphoma, like other cancers, is not infectious and cannot be passed on to other people.
The first sign of the condition may be a painless swelling in the neck, armpit, or groin caused by enlarged lymph nodes. Sometimes more than one group of nodes is affected. The lymphoma may spread to various organs in the body, such as the liver, lungs or bones. 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.
Other 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 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).
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.
Nodal marginal zone B-cell lymphoma is a low-grade lymphoma and often develops very slowly.
As nodal marginal zone B-cell lymphoma tends to progress slowly, it may not be necessary to give treatment immediately if the lymphoma is causing no symptoms. You will, however, be seen regularly by a cancer specialist, and treatment will be recommended if symptoms arise.
Chemotherapy is the use of anti-cancer (cytotoxic) drugs to destroy cancer cells. Nodal marginal zone B-cell lymphoma is usually sensitive to chemotherapy and often disappears with simple drug treatment. This is frequently given in the form of tablets of a drug called chlorambucil (pronounced claw ram bu cil). A drug called fludarabine (flu-dar-a-been) may be used, and this can be given either by injection into a vein or as tablets.
Radiotherapy is the use of high-energy rays to destroy cancer cells, while doing as little harm as possible to the healthy cells. It is usually given if the lymphoma cells are contained in one or two areas of lymph nodes in the same part of the body (stage 1 or 2). It may also be given in addition to chemotherapy.
Although treatment can lead to there being no evidence of the lymphoma being present in the body (remission), it can come back again. However, it may be years before it reappears. Chemotherapy can then be given again and a further remission often occurs. This means that for many patients their disease is controlled, and they can lead a relatively normal life for many years, with some people having a normal life expectancy.
High-dose chemotherapy with bone-marrow or stem-cell infusions may, rarely, be used in some patients whose lymphoma comes back. Your doctor will discuss if this is a suitable treatment for you. This type of treatment involves having very intensive chemotherapy and sometimes radiotherapy.
As the 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.
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 nodal marginal zone B-cell lymphoma are being researched all the time, and you may be invited by your doctor to take part in a research trial to compare a new treatment against the best available standard treatment. Your doctor must discuss the treatment with you, and have your consent before entering you into any clinical trials.
The need for practical and emotional support will of course be individual. For some people with nodal marginal zone B-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 nodal marginal zone B-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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