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:

nodal marginal zone lymphoma (NMZL)
extranodal MZL, called mucosa-associated lymphoid tissue (MALT) lymphoma
splenic marginal zone lymphoma (SMZL).

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.

Related pages

What is cancer?

For healthcare professionals

If you are a healthcare professional, use our guide to find the right information and support for your patients affected by lymphoma. This explains the support available from Macmillan and from other trusted organisations.

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).

Related pages

Do I have cancer?

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.

Related pages

Booklets and resources

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:

R-bendamustine – rituximab and bendamustine

R-chlorambucil – rituximab and chemotherapy tablets called chlorambucil.

R-CHOP

R-CVP

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.

Related pages

After treatment for nodal marginal zone lymphoma

People often have many different feelings when they finish lymphoma treatment. You may feel relieved that treatment has finished, but worried about what will happen in the future.

You will have appointments with your lymphoma doctor or nurse less often than before. But at the same time, you may have new challenges to cope with and things to think about.

We have information below about some of the things people ask about after lymphoma treatment. But you may have other questions or need information about something else. If there is something you want to talk about at any point after treatment, you can:

call the Macmillan Support Line free on 0808 808 00 00
chat to our specialists online
visit our Online Community to talk to people who have been affected by lymphoma, share your experience, and ask an expert your questions.

Related pages

After treatment

Lymphoma follow-up

You will have regular follow-up appointments after your treatment. You can talk to your doctor or nurse about any concerns you 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.

Related pages

Follow-up after lymphoma treatment

Side effects of lymphoma treatment

You may have some ongoing side effects as you recover from lymphoma treatment. You can use our impacts of cancer A-Z to search for information about managing different symptoms and side effects. Or find out more about side effects of treatment for Hodgkin lymphoma or treatment for non-Hodgkin lymphoma.

Tiredness and fatigue

Tiredness (fatigue) often affects people with cancer. It can be caused by lymphoma or be a side effect of lymphoma treatment. RESTORE is an online resource that aims to help people living with cancer related fatigue.
Sexual well-being

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

fertility after treatment for women

fertility after treatment for men.

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.

Well-being and recovery

It can take time to recover after lymphoma treatment. Some days you may feel better than others.

It is important to know where to get support or information if you need it. People often need support even some time after lymphoma treatment. But sometimes it is difficult to know who to ask for help. To find support:

ask your GP or someone from your cancer team for advice about support in your area
search cancercaremap.org to find cancer support services near you
call us free on 0808 808 0000 or talk to us online - our cancer information and support specialists can offer guidance and help you find what you need.

Our course Help to Overcome Problems Effectively (HOPE) helps people during and after cancer treatment. It is a free, interactive, group based, self management support course. It runs for 6 weeks, with each weekly session lasting 2.5 hours. To find out more about HOPE courses in your area, email ServiceOpsSupport@macmillan.org.uk

A healthy lifestyle can help speed up your recovery. Even small lifestyle changes may improve your well-being and long-term health.

Related pages

Being physically active after treatment

Booklets and resources

Support for people with nodal marginal zone lymphoma

We understand that being diagnosed with cancer can affect you both physically and emotionally. We have information and advice to help you.

Talk to someone about nodal marginal zone lymphoma

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:

Call the Macmillan Support Line on 0808 808 00 00.
Chat to our specialists online.
Visit our non-Hodgkin lymphoma forum to talk with people who have been affected by non-Hodgkin lymphoma (NHL), share your experience, and ask an expert your questions.

More information and advice

We know cancer can affect you physically, emotionally and financially. We have information and advice about different ways cancer might impact you, such as help with:

Other organisations who offer information and support

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 provides high quality information and support for people affected by lymphoma. It provides helpline services and a range of peer support including online support meetings, educational events and webinars. Its website includes TrialsLink, a database of lymphoma clinical trials.

Supporting someone with lymphoma

When someone you know is diagnosed with lymphoma, it can be difficult to know how to support them. You may want information to help you understand what they are going through. Or you may be worried about what to say.

We have information to help with the practical, emotional and financial impacts of supporting someone. You can also talk to us by:

calling the Macmillan Support Line
using chat online
visiting our family and friends forum to talk with people who are supporting someone with cancer, share your experience, and ask an expert your questions.

Booklets and resources

Access our lymphoma information in other formats

We are working to make our website as accessible as possible. We want everyone to be able to use it to find the information they need. We have tips about using settings on your computer or device to help you use our website in our accessibility statement.

We also provide information in a range of languages and formats. If you cannot find the information you are looking for in the format or language you need, email us at cancerinformationteam@macmillan.org.uk

Order our non-Hodgkin lymphoma booklet

Download our lymphoma booklet and ebooks

Our Understanding non-Hodgkin lymphoma booklet is available as a pdf to download or view online and in ePub or Mobi formats to download.

Booklets and resources

Listen to our lymphoma audiobook

You can listen to our Understanding non-Hodgkin lymphoma audiobook. You can also search our full range of audiobooks.

Booklets and resources

Find non-Hodgkin lymphoma information in your language

We have a range of translated cancer information. This includes information about different cancer types, being diagnosed, cancer treatment, and side effects. We have some lymphoma information in the following languages. You can also search our most up to date list of web pages we have translated on request.

Bulgarian - Mantle cell lymphoma / Мантелноклетъчен лимфом [PDF]
German - Follicular lymphoma / Follikuläres Lymphom (FL) [PDF]
Polish - Diffuse large B-cell lymphoma (DLBCL) / Chłoniak rozlany z dużych komórek B [PDF]
Polish - Lymphoma / Chłoniak [PDF]
Slovak - Anaplastic large cell lymphoma (ALCL) / Anaplastický veľkobunkový lymfóm [PDF]
Slovak - Follicular lymphoma / Folikulový lymfóm [PDF]
Tamil - Follicular lymphoma [PDF]

If you would like any of our lymphoma information translated into your language, please email cancerinformationteam@macmillan.org.uk

Watch British sign language videos

You can watch our BSL videos about cancer on YouTube.

Find our easy read booklets

Our easy read booklets use simple words and pictures to tell you about cancer. They can be useful if you want information that is easier to understand.

Looking for large print, Braille or another format?

If you would like our information in a different format such as large print or Braille, email us at cancerinformationteam@macmillan.org.uk or call us free on 0808 808 00 00.

About our information

References

Below is a sample of the sources used in our nodal marginal zone lymphoma (NMZL) information. If you would like more information about the sources we use, please contact us at cancerinformationteam@macmillan.org.uk

Thieblemont C, et al. Optimizing therapy for nodal marginal zone lymphoma. Blood. 2016. 127:17; 2064-2071. Available from: www.ashpublications.org/blood/article/127/17/2064/34944/Optimizing-therapy-for-nodal-marginal-zone

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.