Marginal zone lymphomas are B-cell lymphomas. They are not very common and account for a small percentage of non-Hodgkin lymphomas (NHLs). There are 3 main types of marginal zone lymphomas:
Nodal marginal zone lymphoma is very rare. It accounts for about 1–2% of all cases of NHL. Nodal marginal zone lymphoma may also be called monocytoid B-cell lymphoma.
Nodal marginal zone lymphoma is more common in older adults, usually in their 60s or older. It is slightly more common in women than it is in men.
Nodal marginal zone lymphoma usually only involves the lymph nodes. Sometimes it can also be found in the bone marrow. Some people have widespread disease at the time of diagnosis.
Nodal marginal zone lymphoma is often slow growing (indolent). However, it can change (transform) into a more aggressive large cell lymphoma. Nodal marginal zone lymphomas behave slightly more aggressively than MALT lymphomas.
The best treatment for nodal marginal zone lymphoma is not really known. Treatment options for nodal marginal zone lymphoma are often similar to follicular lymphoma.
Because nodal marginal zone lymphoma is a slow-growing (indolent) lymphoma, people may not need treatment right away unless they have symptoms.
Nodal marginal zone lymphoma is usually sensitive to chemotherapy. It often disappears after chemotherapy treatment. Drugs that may be used on their own include:
Combinations of chemotherapy drugs that may also be used include:
Monoclonal antibodies are a type of biological therapy that is effective in treating certain types of NHL. Rituximab (Rituxan) may be used and may be effective for nodal marginal zone lymphoma.
A very small number of people with nodal marginal zone lymphoma will also have hepatitis C infection (HCV). Interferon alfa (Intron A, Wellferon) may be used alone or in combination with a drug called ribavirin, which is used to treat some viral infections like HCV and may be helpful.
External beam radiation therapy may be used to treat the lymph nodes in early stage (stage I or stage II) nodal marginal zone lymphoma, when the lymphoma is contained in 1 or 2 lymph node areas.
A stem cell transplant (SCT) is rarely used. It may be used in some people with nodal marginal zone lymphoma when their lymphoma recurs after treatment.
For more detailed information on specific drugs, go to sources of drug information.
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