Nodal marginal zone b-cell lymphoma (NMZL)
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What is nodal marginal zone lymphoma?
Nodal marginal zone lymphoma (NMZL) is a rare type of blood cancer.
It develops when blood cells called lymphocytes become abnormal. These blood cells normally help fight infection.
The abnormal lymphocytes usually build up in an area of the lymph nodes called the marginal zone.
Other types of marginal zone lymphoma
NMZL is 1 of 3 types of marginal zone lymphoma. The other 2 types are:
- extranodal marginal zone lymphoma – also called mucosa-associated lymphoid tissue (MALT) lymphoma
- splenic marginal zone lymphoma (SMZL).
If you are still having tests or waiting for a clear diagnosis, your doctor may talk to you about all 3 types.
Related pages
Booklets and resources
Symptoms of NMZL
B symptoms
NMZL may also cause the following symptoms:
- drenching night sweats
- unexplained weight loss.
These are called B symptoms. Along with staging and grading (below), B symptoms can help your cancer team understand:
- how the lymphoma might develop
- which treatments might help.
Worried about symptoms?
A change in how you feel or how your body works can be caused by conditions other than NMZL. But always ask your GP to check any ongoing, new or unexplained symptoms.
We are also here if you need someone to talk to. You can:
- call the Macmillan Support Line free on 0808 808 00 00
- chat to our specialists online.
Related pages
Causes of NMZL
The causes of 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 it is most common in people over the age of 50.
Like other cancers, NMZL is not infectious. You cannot spread it to other people.
We have more information about the causes and risk factors for non-Hodgkin lymphoma.
Diagnosis of NMZL
If you have symptoms, you should start by going to your GP. They may arrange for you to have blood tests or scans. If they think cancer may be causing your symptoms, they will refer you to a hospital for tests and for specialist advice and treatment.
At the hospital, the doctor will ask about any symptoms, your general health and any illnesses you have had. They will also examine you.
The most important test for diagnosing lymphoma is a biopsy. A biopsy means testing a small piece of tissue or a sample of cells from an affected area of the body. Sometimes NMZL can be found with a blood test.
Find out more about having a biopsy and other tests in our general general lymphoma information.
Waiting for test results
Waiting for test results can be a difficult time. It may take up to 2 weeks for the results to be ready.
You may find it helpful to talk to family members or a close friend. Your specialist nurse can also provide support.
You can talk to one of our cancer support specialists for free on 0808 808 0000, 7 days a week, 8am to 8pm.
Related pages
Booklets and resources
Staging and grading of NMZL
Your test results give your cancer team more information about the lymphoma. This includes:
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The stage
Staging describes how many areas of the body are affected by lymphoma and where these areas are.
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The grade
Grading describes how and how quickly the lymphoma cells are likely to grow. NMZL is low grade lymphoma. This means it usually develops slowly. Sometimes it can change (transform) into high grade lymphoma. High grade lymphoma grows more quickly and needs treatment straight away.
Knowing the stage and grade of the lymphoma helps your cancer team plan the right treatment for you.
We have more information about staging and grading non-Hodgkin lymphoma.
Treatment for NMZL
A team of specialists will meet to discuss the best possible treatment for you. They are called a multidisciplinary team (MDT).
After the MDT meeting, you will usually meet with your cancer doctor and specialist nurse. They will explain the different treatments and their side effects. They will also talk to you about things to consider when making treatment decisions.
Your treatment options will depend on:
- the stage of the lymphoma
- your general health
- your preferences.
If you have no symptoms, you may not need any treatment. Instead, you may have regular tests and appointments to monitor the lymphoma and check for signs that you need to start treatment. This is called watch and wait. It may also be called active surveillance or active monitoring. It is a way of safely delaying treatment until you need it.
If you need to start treatment, it may include one of the following. You may have some treatments as part of a clinical trial.
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Cancer drug treatment
NMZL is often treated with a combination of the immunotherapy drug rituximab and chemotherapy. You may have rituximab on its own. Commonly used combinations include:
- rituximab with bendamustine
- R-CVP
- R-CHOP.
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Radiotherapy
Radiotherapy uses high-energy rays called radiation to treat cancer. It destroys cancer cells in the area you have it.
You might have radiotherapy as a first treatment if the lymphoma is only in 1 or 2 groups of lymph nodes in the same part of the body. This is called stage 1 or 2 lymphoma. In some people, this may cure the lymphoma. NMZL is usually very sensitive to radiotherapy.
Treating NMZL relapse
Treatment for NMZL is often very effective. It may make all signs of lymphoma disappear for a time. Some people will not need further treatment for many years. But NMZL often comes back. This is called recurrence or relapse.
If you have a relapse with no symptoms, you may have ongoing monitoring with tests and scans. If you need to start treatment, it may involve the following:
- radiotherapy to treat an area of lymphoma that is causing symptoms
- rituximab
- rituximab with chemotherapy
- intensive treatment with a stem cell transplant
- targeted therapy drugs, such as zanubrutinib.
Your cancer team will talk to you about the best options for your situation.
Treating transformed NMZL
Sometimes NMZL can change from low grade into high grade lymphoma. This is called transformation or transformed NMZL. It is not common. But your cancer team will do tests to check for this when you are diagnosed or have a relapse of NMZL.
High grade lymphoma grows more quickly. It is treated differently. Transformed NMZL is treated in the same way as another type of lymphoma called DLBCL.
After NMZL treatment
Follow-up after treatment for NMZL
You will have regular check-ups during and after your NMZL treatment. Your cancer doctor or specialist nurse will explain what to expect.
Follow-up appointments are a good time for you to talk to your cancer doctor or specialist nurse about any concerns you have. Tell them as soon as possible if you have any problems or notice new symptoms between appointments. We have more information about follow-up care after treatment.
Sometimes side effects may continue or develop months or years after treatment. These are called late effects. We have more information about late effects of lymphoma treatment.
Wellbeing and recovery
Looking after yourself can help speed up your recovery after NMZL treatment. Even small lifestyle changes may improve your wellbeing and long term health.
Even if you already have a healthy lifestyle, you may choose to make some positive changes after treatment. We have more information on leading a healthy lifestyle after treatment.
Support for people with NMZL
People often have many different feelings during and after NMZL treatment. If you have finished treatment, you may feel relieved it has ended, but worried about what might happen in the future.
It is important to know where to get support or information if you need it. People often need support even some time after NMZL treatment. But sometimes it is difficult to know who to ask for help.
You can:
- ask your GP or someone from your cancer team for advice about support in your area
- find cancer support services near you
- call the Macmillan Support Line for free on 0808 808 00 00.
- chat to our cancer information specialists online.
- visit our non-Hodgkin lymphoma forum to talk to people who have been affected by lymphoma, share your experience, and ask your questions.
You may also find the HOPE programme helpful. The programme is a free 6 week self-management course designed to help you develop techniques and strategies when living with or after cancer. Topics include goal setting, fatigue management, and wellbeing.
You can sign up for the online HOPE programme. Or to find out more about face to face programmes in your area, email ServiceOpsSupport@macmillan.org.uk
Other organisations who offer information and support
- Blood Cancer UK offers support and information to people affected by blood cancers, including lymphoma.
- 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.
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:
About our information
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.
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References
Below is a sample of the sources used in our lymphoma information. If you would like more information about the sources we use, please contact us at informationproductionteam@macmillan.org.uk
Cwynarski K, Marzolini MAV, Barrington SF, et al. The management of primary mediastinal B-cell lymphoma: a British Society for Haematology Good Practice Paper. Br J Haematol. 2019;185:402-9.
Eyre TA, Bishton MJ, McCulloch R, et al. Diagnosis and management of mantle cell lymphoma: A British Society for Haematology Guideline. Br J Haematol. 2024;204(1):108–26.
Ferreri AJM, Illerhaus G, Doorduijn K, et al., on behalf of the EHA and ESMO Guidelines Committees. Primary central nervous system lymphomas: EHA–ESMO Clinical Practice Guideline for diagnosis, treatment and follow-up. Ann Oncol. 2024;35(6):491-507.
Fox CP, Ahearne MJ, Pettengell R, et al. Guidelines for the management of mature T- and natural killer-cell lymphomas (excluding cutaneous T-cell lymphoma): a British Society for Haematology Guideline. Br J Haematol. 2022;196:507-22.
Fox CP, Chaganti S, McIlroy G, et al. The management of newly diagnosed large B-cell lymphoma: A British Society for Haematology Guideline. Br J Haematol. 2024;204(4):1178–92.
McNamara C, Montoto S, Eyre TA, et al. The investigation and management of follicular lymphoma. Br J Haematol. 2020;191:363-81.
Pratt G, El-Sharkawi D, Kothari J, et al. Diagnosis and management of Waldenström macroglobulinaemia—A British Society for Haematology guideline. Br J Haematol. 2022;197:171–87.
Walewska R, Eyre TA, Barrington S, et al. Guideline for the diagnosis and management of marginal zone lymphomas: A British Society of Haematology Guideline. Br J Haematol. 2024;204(1):86–107.
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