What is nodal marginal zone lymphoma?

Nodal marginal zone lymphoma (NMZL) is a rare type of non-Hodgkin lymphoma (NHL).

It belongs to a group of non-Hodgkin lymphomas called marginal zone lymphomas (MZL). There are three types:

Your doctor may talk to you about the other marginal zone lymphomas before it is clear which type of MZL you have.

NMZL develops when B-cells become abnormal (cancerous). B-cells are white blood cells that normally help fight infection. They are sometimes called B-lymphocytes.

The abnormal B-cells usually build up in lymph nodes. This is why NMZL is described as nodal. The marginal zone is an area of lymph nodes. This is where abnormal B-cells are found in NMZL.

Symptoms of nodal marginal zone lymphoma

Painless swelling in the neck, armpit or groin

This is the most common sign of NMZL. It is caused by lymphoma cells building up in the lymph nodes, which makes them bigger.

B symptoms

Some people also have symptoms that doctors call B symptoms. These can include:

  • drenching night sweats which require a change of nightwear and bed covers
  • high temperatures (fevers) with no obvious cause
  • unexplained weight loss.

Knowing if you have any B symptoms will help your doctor to stage the lymphoma and plan your treatment.

Other symptoms

General symptoms of NMZL may include tiredness (fatigue)If the NMZL has affected the bone marrow, tiredness may be caused by a low number of red blood cells (anaemia).

Causes of nodal marginal zone lymphoma

The causes of nodal marginal zone lymphoma (NMZL) are mostly unknown. It is sometimes linked to long-term infection with the hepatitis C virus (HCV). But it is important to remember that most people with HCV will not develop lymphoma.

NMZL can affect people of all ages but is most common in people over the age of 50.

Like other cancers, NMZL is not infectious. It cannot be passed on to other people.

We have more information about causes and risk factors for non-Hodgkin lymphoma.

Diagnosis of nodal marginal zone lymphoma

The most common test for diagnosing lymphoma is a biopsy. A doctor will take a sample of tissue from the affected area. The most common place to take a biopsy from is an enlarged lymph node. This is called a lymph node biopsy). You may have all or a part of the lymph node removed. The tissue will be sent to a laboratory for testing. You may also have biopsies taken from other areas of your body.

You can read more about further tests you may have in our information about non-Hodgkin lymphoma.

Waiting for test results can be a difficult. You may find it helpful to talk to your family, friends or specialist nurse.

The stages and grades of nodal marginal zone lymphoma

Your test results will help your doctors find out how many areas of your body are affected by lymphoma and where these areas are. This is called staging.

Lymphomas are also grouped as either low-grade or high-grade. Low-grade lymphomas are usually slow growing. High-grade lymphomas usually grow more quickly. Nodal marginal zone lymphoma (NMZL) is a low-grade lymphoma and normally develops slowly. Sometimes it can change (transform) to become a high-grade lymphoma.

Knowing the stage of the lymphoma helps your doctor plan the right treatment for you.

We have more information about the stages and grades of non-Hodgkin lymphoma.

Treatment for nodal marginal zone lymphoma

A team of specialists will meet to discuss the best possible treatment for you. They are called a multidisciplinary team (MDT).

Your doctor, cancer specialist or nurse will explain the different treatments and their side effects to you. They will also talk to you about things to consider when making treatment decisions.

Treatment for NMZL depends on the stage of the lymphoma. It also depends on if you have symptoms that are causing problems.

NMZL usually develops slowly. Some people may not need treatment for months or years. During this time, they will have regular check-ups to monitor the lymphoma. This is called watch and wait.

If treatment is needed, the aim is to control the lymphoma for as long as possible. This is called remission.

The most common treatments for NMZL include:

  • Immunotherapy and chemotherapy

    NMZL is often treated with a combination of the immunotherapy drug rituximab and chemotherapy. This is called chemoimmunotherapy. Sometimes rituximab is used on its own. Commonly used combinations of chemoimmunotherapy include:

    There are other chemotherapy drugs and combinations that can be used to treat NMZL. Your lymphoma doctor can tell you which ones are most appropriate for you.

  • Steroids

    Steroids are drugs that are often given with chemotherapy to treat lymphomas. They help make chemotherapy more effective.

  • Radiotherapy

    Radiotherapy uses high-energy rays to destroy cancer cells, while doing as little harm as possible to healthy cells. It may be used as a first treatment if the lymphoma cells are contained in 1 or 2 groups of lymph nodes in the same part of the body. This is called stage 1 or 2. In some people, this may cure the lymphoma. NMZL is usually very sensitive to radiotherapy.

    Radiotherapy can also be used to treat lymphoma that has come back in one area of lymph nodes. It may sometimes be used to treat symptoms, such as pain.

  • Maintenance treatment

    You may be offered treatment to help stop NMZL coming back. This is called maintenance treatment.

You may have some treatments as part of a clinical trial.

If the lymphoma comes back

If NMZL comes back after treatment it is called a recurrence or relapse. If this happens, you can usually have more treatment to help control it again.

A stem cell transplant may be used if the lymphoma comes back after treatment. A stem cell transplant is an intensive treatment, so it is not suitable for everyone. Very few people with NMZL will have a stem cell transplant.

We have more information about treating lymphoma that has come back.

After treatment for nodal marginal zone lymphoma

You will have regular follow-up appointments after your treatment. You can talk to your doctor or nurse about any concerns you may have at these appointments. Your doctor will want to know how you are feeling, and to check you are recovering from any side effects of treatment.

Late effects

Sometimes side effects may continue or develop months or years after treatment. These are called late effects. We have more information about long-term and late effects of treatment for lymphoma.

Sex life and fertility

Cancer and its treatment can sometimes affect your sex life. There ways to improve your sexual well-being and to manage any problems.

Treatment for lymphoma may affect your fertility. If you are worried about your fertility it is important to talk with your doctor before you start treatment. We have more information about:

Well-being and recovery

Even if you already have a healthy lifestyle, you may choose to make some positive lifestyle changes after treatment.

Making small changes such as eating well and keeping active can improve your health and wellbeing and help your body recover.

Getting support

Everyone has their own way of dealing with illness and the different emotions they experience. You may find it helpful to talk things over with family and friends or your doctor or nurse.

Macmillan is also here to support you. If you would like to talk, you can:

The organisations below also offer information and support:

  • Blood Cancer UK

    Blood Cancer UK offers support and information to people affected by blood cancers, including lymphoma.

  • Lymphoma Action

    Lymphoma Action gives emotional support, advice and information for people with Hodgkin lymphoma or non-Hodgkin lymphoma and those close to them. It has a national network of people with lymphoma, as well as local groups. Their website has a section called trialslink where you can see information about lymphoma clinical trials.

About our information

  • Reviewers

    This information has been written, revised and edited by Macmillan Cancer Support’s Cancer Information Development team. It has been reviewed by expert medical and health professionals and people living with cancer. It has been approved by Senior Medical Editor, Professor Rajnish Gupta, Macmillan Consultant Medical Oncologist.

    Our cancer information has been awarded the PIF TICK. Created by the Patient Information Forum, this quality mark shows we meet PIF’s 10 criteria for trustworthy health information.

Reviewed: 31 January 2021
Reviewed: 31/01/2021
Next review: 31 January 2024
Next review: 31/01/2024