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Nodal marginal zone lymphoma

Nodal marginal zone lymphoma is a type of marginal zone lymphoma. It is rare- accounting for about 1-2% of all non-Hodgkin lymphoma cases.

Marginal zone lymphomas are rare types of non-Hodgkin lymphoma (NHL). There are 3 main types of marginal zone lymphomas: extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT); splenic marginal zone lymphoma; and nodal marginal zone lymphoma*. Nodal marginal zone lymphoma (NMZL) is often referred to as ‘monocytoid B-cell lymphoma’ and is usually slow-growing. Whilst it is unlikely, NMZL does have the ability to transform into a more aggressive type of lymphoma.

How does Nodal marginal zone lymphoma affect the body?

NMZL generally only involves the lymph nodes, with the most common symptom usually being a painless swelling in the neck, armpit or groin caused by enlarged lymph nodes. More than one group of nodes may be affected, and NMZL can sometimes be found in the bone marrow. It is not uncommon for people with NMZL to have widespread disease at the time of diagnosis, where the disease has spread to several areas of the body.

Who does Nodal marginal zone lymphoma commonly affect?

NMZL usually affects adults aged 60 years and over. It is slightly more common in women than it is in men.

Do we know what causes Nodal marginal zone lymphoma?

Often, treatment for NMZL may not be required immediately. Unless symptoms are present, most people are managed by the ‘watch and wait’ approach which involves regular monitoring of the condition by their doctor or specialist*.  Blood tests and imaging investigations as well as clinical examination are performed. Once symptoms develop, or if the NMZL is progressing, chemotherapy is used to treat the lymphoma. For localised disease, radiotherapy may be useful. Single agent chemotherapy drugs such as fludarabine and chlorambucil may also be used to treat NMZL. Commonly used combination chemotherapy regimens for NMZL include: CVP (cyclophosphamide, vincristine and prednisolone); CHOP (cyclophosphamide, doxorubicin, vincristine and prednisolone), and F+C (fludarabine and cylcophosphamide). Rituximab, a monoclonal antibody commonly used to treat lymphoma, is often used in combination with these chemotherapy agents.

Whilst rarely used, stem cell transplants may be an option for some people with relapsed nodal marginal zone lymphoma.

+Not approved by the Therapeutic Goods Administration for this indication in Australia. May be available in some health facilities through local protocols