Nodal marginal zone lymphoma

Nodal marginal zone lymphoma is a type of non-Hodgkin lymphoma. It develops from abnormal B-lymphocytes – the lymphoma cells. (B-lymphocytes are white blood cells that fight infection.) The lymphoma cells build up in lymph nodes.

The most common symptom is a painless swelling in the neck, armpit or groin. Other symptoms may include: tiredness, weight loss, night sweats and high temperatures (fevers).

To diagnose NMZL a doctor removes an enlarged lymph node and checks it for lymphoma cells. You will also have tests and scans to find out how many groups of lymph nodes are affected and whether the lymphoma has spread elsewhere. This is called staging.

NMZL is slow-growing and doesn’t always need to be treated straight away. When treatment is needed this is usually with chemotherapy often combined with a monoclonal antibody drug called rituximab. Some people may have radiotherapy. You may be invited to join a clinical trial looking at new ways of treating NMZL.

Nodal marginal zone lymphoma (Nodal MZL)

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

This section should ideally be read with our general information about NHL. We hope this information answers your questions. If you have any further questions, you can ask your doctor or nurse at the hospital where you are having treatment.

Non-Hodgkin lymphoma

Non-Hodgkin lymphoma (NHL) is a cancer of the lymphatic system. The lymphatic system is part of the body’s immune system and helps us fight infection. The lymphatic system includes organs such as the bone marrow, thymus, spleen, and the lymph nodes (or lymph glands).

The lymphatic system
The lymphatic system

View a large version

Read a description of this image

Lymph nodes are connected by a network of tiny lymphatic vessels that contain lymph fluid. There is also lymphatic tissue in other organs, such as the skin, lungs and stomach. There are lymph nodes all over the body. As lymph fluid flows through the lymph nodes, the nodes collect and filter out anything harmful or anything the body doesn't need. This includes bacteria, viruses, damaged cells and cancer cells.

Lymph fluid contains lymphocytes. These are a type of white blood cell that helps the body fight infection and disease.

Lymphocytes start to grow in the bone marrow, where blood cells are made. The two main types of lymphocyte are B-cells and T-cells. B-cells mature in the bone marrow, while T-cells mature in the thymus gland behind the breast bone. When they're mature, both B-cells and T-cells help fight infections. Lymphoma is a disease in which either T-cells or B-cells grow in an uncontrolled way.

There are many different types of NHL. They're grouped (or classified) according to certain characteristics. The most widely used classification system is produced by the World Health Organisation.

What is Nodal marginal zone lymphoma (nodal MZL)

Nodal marginal zone lymphoma (nodal MZL) is a rare cancer of the B-cell lymphocytes. It usually affects people in their 60s and older.

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 nodal MZL.

Causes of nodal MZL

The causes of nodal MZL are unknown. Like all cancers, it isn’t infectious and cannot be passed on to other people. It is not inherited and can’t be passed onto children.

Signs and symptoms of nodal MZL

The first sign is often a painless swelling in the neck, armpit or groin, caused by enlarged lymph nodes. Other symptoms may include loss of appetite and tiredness (fatigue).

Some people have night sweats, unexplained high temperatures (fevers) and weight loss. These are known as B symptoms

How nodal MZL is diagnosed

A diagnosis is made by removing an enlarged lymph node (a biopsy) and examining the cells under a microscope.

It is a very small operation and may be done under local or general anaesthetic. Biopsies may also be taken from other areas of the body.
Doctors may also want to look closely at the stomach (using a long flexible tube called an endoscope) to see if the lymphoma has started there. This is because it is more common for marginal zone lymphoma to begin in the stomach than in the lymph nodes. Nodal marginal zone lymphoma is only diagnosed if there is no sign of lymphoma in the stomach. 

Additional tests, including blood tests, x-rays, scans and bone marrow samples, may be used to find out more about the type of lymphoma and how far it has spread in the body. Doctors use this information to decide which treatment is most appropriate for you.

You can read more about these tests in our information about non-Hodgkin lymphoma.

Staging and grading of nodal MZL


The stage of non-Hodgkin lymphoma describes how many groups of lymph nodes are affected, where they are in the body, and whether other organs such as the bone marrow or liver are involved. A group of lymph nodes refers to lymph nodes in one area of the body, such as in the armpit, on one side of the neck, or in the groin.

Stage 1

One group of lymph nodes is affected.

Stage 2

Two or more groups of lymph nodes are affected and they are all either above or below the diaphragm (the sheet of muscle under the lungs that separates the chest from the abdomen).

Stage 3

The lymphoma is in lymph nodes both above and below the diaphragm.

Stage 4

The lymphoma has spread to other organs, such as the bone marrow, liver or lining of the lungs (pleura).

B symptoms

Doctors also add either the letter A or B to show whether you have any of the following symptoms: weight loss, fevers or night sweats.

If you don't have any of these symptoms, the letter A will be added next to the stage. If you do have these symptoms, the letter B is added next to the stage.


Non-Hodgkin lymphomas are divided into two groups: indolent (sometimes called low-grade) and aggressive (sometimes called high-grade). Indolent lymphomas are usually slow-growing and aggressive lymphomas grow more quickly.

Nodal MZL is an indolent lymphoma and usually develops very slowly. In some people, it may change (transform) into an aggressive non-Hodgkin lymphoma, which means it will need more intensive treatment.

Treatment for nodal MZL

If the lymphoma is not causing symptoms, you may not need treatment immediately. Delaying treatment allows you to avoid side effects for as long as possible and doesn’t have any effect on how well treatment works.

You will be seen regularly by your cancer specialist and you will be advised to have treatment if you develop symptoms.

After treatment, many people have a period of time with no signs of active disease known as remission. If the lymphoma comes back, it can be treated again. This can give another period of remission and the lymphoma can often be controlled in this way for many years.


Chemotherapy is the use of anti-cancer (cytotoxic) drugs to destroy cancer cells. It is an important treatment for nodal MZL and can often get the lymphoma into remission.

Chemotherapy may be given with a drug called rituximab (Mabthera ®), which is a monoclonal antibody that targets B-cells.

Chemotherapy can be given as tablets or into a vein (intravenously). You may be given just one type of chemotherapy drug or you may be given two or more together (combination chemotherapy).

Chemotherapy treatments for nodal MZL include:

Chlorambucil is a commonly used treatment. It comes as tablets.

CVP is a combination of the chemotherapy drugs cyclophosphamide and vincristine, and the steroid prednisolone. It's given as a drip into a vein, usually once every three weeks. Rituximab (Mabthera ®) is often given with CVP. This combination is called R-CVP.

Bendamustine is given into a vein, usually for 2 days every month. It is often given with rituximab.

There are other chemotherapy drugs and combinations that can be used to treat nodal MZL. Your specialist will be able to tell you which treatment is the most appropriate for you.

Monoclonal antibody therapy

Monoclonal antibodies are drugs that recognise, target and stick to specific proteins on the surface of cancer cells, and can stimulate the body’s immune system to destroy these cells.

Rituximab (Mabthera ®)

Rituximab is a monoclonal antibody that targets B-cells. It may be used to treat nodal MZL. It is given as a drip into a vein (intravenous infusion).


Radiotherapy uses high-energy rays to destroy cancer cells, while doing as little harm as possible to nearby healthy cells. It is usually given if the lymphoma cells are contained in one or two areas of lymph nodes in the same part of the body (stage 1 or 2).


Steroids are drugs that are often given with chemotherapy to treat lymphomas. They also help you feel better and can reduce feelings of sickness.

Stem cell treatment (transplants)

Some people with lymphoma may have treatments using their own stem cells or stem cells from a donor. Stem cells are early blood cells that develop into all the other types of blood cell. This treatment is not suitable for everyone and is not done routinely. Doctors take into account a person's general health and age before recommending them.

Some people have some of their own stem cells collected and stored. This allows them to have higher doses of chemotherapy to destroy the lymphoma cells. After the chemotherapy, their stem cells are returned by a drip (like a blood transfusion) to help their blood cells recover from the effects of chemotherapy. This is called high-dose chemotherapy with stem cell support.

Some people may have treatment using stem cells from another person (a donor). This is called a donor (allogeneic) stem cell transplant.

Clinical trials

New treatments for nodal marginal zone lymphoma are being researched all the time

Your doctor may invite you to take part in a clinical trial to compare a new treatment against the best available standard treatment. They must discuss the treatment with you and have your informed consent before entering you into a trial.

Before any trial is allowed to take place, it must be approved by a research ethics committee that protects the interests of those taking part.

You may decide not to take part, or to withdraw from the trial at any stage. You will then receive the best standard treatment available.

Your feelings

You may have many different emotions including anger, resentment, guilt, anxiety and fear. These are all normal reactions, and are part of the process many people go through in trying to come to terms with their condition.

Everyone has their own way of coping with difficult situations. Some people find it helpful to talk to family or friends, while others prefer to seek help from people outside their situation. Some people prefer to keep their feelings to themselves. There is no right or wrong way to cope, but help is there if you need it. Our cancer support specialists can give you information about counselling in your area.

Other useful organisations

The Lymphoma Association

The Lymphoma Association gives emotional support, advice and information on all aspects of Hodgkin lymphoma and non-Hodgkin lymphoma. It has a national network of people with lymphoma and local groups.

Leukaemia CARE

Leukaemia CARE is a national group promoting the welfare of people with leukaemia and other blood disorders, including non-Hodgkin lymphoma. Has regional support groups in many counties.