Nodal marginal zone lymphoma

Nodal marginal zone lymphoma is a rare, slow-growing (indolent) B-cell non-Hodgkin lymphoma (NHL). It can change into a fast-growing (aggressive) type of NHL.

Nodal marginal zone lymphoma usually only affects the lymph nodes. In rare cases, it may develop in other organs outside of the lymph nodes (called extranodal disease). Sometimes it can develop in the spleen or bone marrow.

Nodal marginal zone lymphoma is more common in older adults. People with this disease are usually diagnosed when they are 60 years old or older. It is more common in women than in men. More than 70% of people are stage 3 or 4 at the time of diagnosis.

Most people with nodal marginal zone lymphoma don’t have any symptoms. Some people have B symptoms, which are unexplained fever, drenching night sweats and unexplained weight loss.

Experts don’t really know how best to treat nodal marginal zone lymphoma. You may be offered one or more of the following treatments.

Watchful waiting (also called active surveillance) may be an option because nodal marginal zone lymphoma develops slowly and may not need to be treated right away. The healthcare team will monitor the person with nodal marginal zone lymphoma carefully and start treatment when symptoms appear or there are signs that the disease is progressing more quickly.

Nodal marginal zone lymphoma usually responds well to chemotherapy. It may be given as a single drug or a combination of drugs and is often given with a targeted therapy drug.

Single drugs that may be used for nodal marginal zone lymphoma are:

• fludarabine (Fludara)
• bendamustine (Treanda)

Combinations of chemotherapy drugs that may be used are:

• CHOP – cyclophosphamide (Cytoxan, Procytox), doxorubicin (Adriamycin), vincristine (Oncovin) and prednisone
• R-CHOP – CHOP with rituximab (Rituxan)
• CVP – cyclophosphamide, vincristine and prednisone
• R-CVP – CVP with rituximab
• BR – bendamustine and rituximab

Targeted therapy uses drugs to target specific molecules (such as proteins) on the surface of cancer cells. These molecules help send signals that tell cells to grow or divide. By targeting these molecules, the drugs stop the growth and spread of cancer cells while limiting harm to normal cells.

Rituximab is a targeted therapy drug commonly used alone or in combination with chemotherapy to treat nodal marginal zone lymphoma.

Ibritumomab (Zevalin) is a type of radioimmunotherapy. This treatment attaches a radioactive material to the targeted therapy drug. The drug attaches to the lymphoma cells so the radiation is delivered directly to them, which may mean fewer or less severe side effects.

Immunotherapy helps to strengthen or restore the immune system’s ability to fight cancer. Interferon alfa (Intron A, Wellferon) may be given to people with nodal marginal zone lymphoma who also have a hepatitis C virus (HCV) infection. Interferon alfa may be used alone or with ribavirin, which is a drug used to treat viral infections like HCV.

External beam radiation therapy may be given to the lymph nodes to treat early stage (stage 1 or stage 2) nodal marginal zone lymphoma. It is used when this type of lymphoma affects only 1 or 2 areas of lymph nodes in the body.