Nodal marginal zone lymphoma (NMZL) is a slow growing, type of non-Hodgkin lymphoma (NHL). It develops when B-cells (also called B-lymphocytes) become abnormal. B-cells are white blood cells that fight infection.
The abnormal B-cells (lymphoma cells) usually build up in lymph nodes, but they can affect other parts of the body.
Nodal means the lymphoma started in the lymph nodes. The marginal zone is an area of the lymph nodes. This is where the abnormal B-cells are found in NMZL.
Painless swelling in 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.
Some people also have:
- drenching night sweats
- high temperatures (fevers) with no obvious cause
- unexplained weight loss
Doctors call this group of symptoms B symptoms.
It is not usually clear why NMZL develops, but some cases may be linked to long-term infection with the virus hepatitis C (HCV). Like other cancers, NMZL is not infectious and cannot be passed on to other people. It is most common in people between the ages of 50 and 64.
We have more information about causes and risk factors for non-Hodgkin lymphoma.
The most common test for this lymphoma is to remove part or all of an enlarged lymph node (a biopsy). This may be done under local or general anaesthetic. The biopsy is then sent to a laboratory to be checked for lymphoma cells.
An endoscopy uses a long, flexible tube with a camera on the end, called an endoscope. A doctor gently puts the tube into your mouth and down your throat into your stomach.
It is more common for marginal zone lymphoma to begin in the stomach than in the lymph nodes. NMZL is only diagnosed if there is no sign of lymphoma in the stomach.
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 time. It may help to talk to your family, friends or specialist nurse.
The results of your tests help your doctors find out how many areas of the 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 and high-grade lymphomas grow more quickly. NMZL is a low-grade lymphoma and usually develops slowly. Sometimes it can change (transform) to become a high-grade lymphoma.
We have more information about staging and grading of non-Hodgkin lymphoma.
A team of specialists will meet to discuss the best possible treatment for you. This is called a multidisciplinary team (MDT).
Your doctor or cancer specialist or nurse will explain the different treatments and their side effects. They will also talk to you about things to consider when making treatment decisions.
NMZL often develops slowly. Many people may not need treatment straight away, or even for months or years. If you do not need immediate treatment, you will have regular check-ups at the hospital. Your lymphoma doctor will examine you and do blood tests. This is watch and wait.
For most people with NMZL, the aim of treatment is to get rid of as much of the lymphoma as possible. If there are no signs of NMZL after treatment, this is called a complete remission.
Treatment depends on:
- the stage of the lymphoma
- whether you have symptoms that are causing problems.
Treatments for NMZL might include the following:
Chemotherapy treatments for NMZL include the following:
- R-chlorambucil – this is made up of rituximab and chemotherapy tablets called chlorambucil.
- R-bendamustine – this is made up of rituximab and the chemotherapy drug bendamustine. This treatment is given as a drip into a vein.
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.
Radiotherapy uses high-energy rays to destroy cancer cells, while doing as little harm as possible to healthy cells. NMZL is usually very sensitive to radiotherapy. 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 (stage 1 or 2). In some people, this may cure the lymphoma. 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.
Stem cell transplants
Stem cell transplant may be used if the lymphoma comes back after treatment. Stem cell transplants are intensive treatments, so are not suitable for everyone. You may have a transplant using your own stem cells (autologous stem cell transplant) or cells from a donor (allogeneic stem cell transplant).
You may have some treatments as part of a clinical trial.
If NMZL comes back after treatment, further treatment can often be successful at getting the lymphoma into remission again.
We have more information about treating lymphoma that has come back.
You have regular follow-up appointments after treatment. These appointments are a good opportunity for you to talk to your doctor or nurse about any concerns you have. Your doctor will want to know how you are feeling generally, and to check you are recovering from any side effects of treatment.
Sometimes a side effect may continue or develop months or years after treatment. This is called a late effect.
We have more information about long-term and late effects of treatment for 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:
The organisations below also offer information and support:
Blood Cancer UK
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 firstname.lastname@example.org
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
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.
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