Nodal Marginal Zone B-Cell Lymphoma Overview

Nodal marginal zone B-cell lymphoma is a type of non-Hodgkin lymphoma (NHL). It has also been called monocytoid B cell lymphoma or MZL.

Lymphomas are cancers of lymph cells, a type of blood cell. There are two main categories—Hodgkin lymphoma and non-Hodgkin lymphoma (NHL).

Nodal marginal zone B-cell lymphoma is one of the uncommon types of NHL. It is a type of low-grade (or slow-growing) lymphoma that arises from B-cells and mainly affects lymph nodes.

In addition, nodal marginal zone B-cell lymphoma mainly affects older individuals. There are no known direct risk factors for this lymphoma but a few risk factors associated with lymphomas in general.

Dividing lymphoma cancer cells
STEVE GSCHMEISSNER / Getty Images

Symptoms

The most common symptom of this lymphoma is the enlargement of lymph nodes. Enlarged nodes become apparent as swellings in the neck, armpits or groin. This may be the only symptom, or there may be other associated symptoms and signs like fever and weight loss.

Diagnosis

All lymphomas are diagnosed with a biopsy. A lymph node biopsy is done by excising an entire lymph node, which is then examined under the microscope. The pattern of cells seen under the microscope and tests for specific lymphoma markers will diagnose it as this particular type of non-Hodgkin lymphoma.

After a lymph node biopsy diagnoses this lymphoma, several tests are performed to find out how much this lymphoma has spread in the body. Tests may include CT scans, PET scans, blood tests, and a bone marrow test. These tests are useful in determining the staging and prognosis of the lymphoma.

Staging and Prognosis

Depending on the extent of lymphoma at diagnosis, the disease is grouped into one of four stages. Most individuals with nodal marginal zone B-cell lymphoma are diagnosed at an early stage (when the lymphoma is restricted to one or a few lymph node areas).

The stage and other factors like age, fitness, and blood test results determine the disease outlook or prognosis. Unless the disease has spread extensively, this lymphoma has a good prognosis with a favorable outcome after treatment.

Treatment

Nodal marginal zone B-cell lymphoma is a slow-growing lymphoma. The treatment of this lymphoma is along the same lines as that of follicular lymphoma, the most common type of low-grade non-Hodgkin lymphoma (NHL).

If there are no symptoms, watching and waiting may be the approach used, with regular monitoring.

A common treatment for this lymphoma is Ritxumab, a monoclonal antibody. While Rituximab can be given together with chemotherapy, it is also frequently given by itself for this type of lymphoma.

The majority of individuals with this lymphoma have a good response to treatment and a chance of long-term disease control.

1 Source
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  1. van den Brand M, van Krieken JH. Recognizing nodal marginal zone lymphoma: recent advances and pitfalls. A systematic review. Haematologica. 2013;98(7):1003–1013. doi:10.3324/haematol.2012.083386

Additional Reading

By Indranil Mallick, MD
 Indranil Mallick, MD, DNB, is a radiation oncologist with a special interest in lymphoma.