Nodal marginal zone B-cell lymphoma

Nodal marginal zone lymphoma (NMZL) is a rare type of non-Hodgkin lymphoma (NHL). It develops in the lymph nodes when the body makes abnormal B-cells – the lymphoma cells. B-cells are white blood cells that fight infection.

The most common symptom is a painless swelling in the neck, armpit or groin. This is caused by the lymphoma cells building up in the lymph nodes, making them bigger.

Other symptoms may include:

  • tiredness
  • unexplained weight loss
  • drenching night sweats
  • 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 about the type of lymphoma and whether it has spread.

NMZL may not need to be treated straight away. Treatment is usually with chemotherapy, but some people may have radiotherapy. Or you may be invited to join a clinical trial looking at new ways of treating NMZL. You can talk to your lymphoma doctor about this.

Nodal marginal zone B-cell lymphoma (NMZL)

It is best to read this information with our general information about non-Hodgkin lymphoma (NHL). If you have any more questions, you can ask your doctor or nurse at the hospital where you are having treatment.

Nodal marginal zone B-cell lymphoma (NMZL) is a slow-growing type of 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.


Causes and risk factors for NMZL

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 lymphoma 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 risk factors for lymphoma.


Signs and symptoms of NMZL

Many people don’t have symptoms in the early stages of NMZL. It may be discovered by chance when a test is done or a treatment is given for another reason.

The first sign of NMZL is often a painless swelling in the neck, armpit or groin.

This is caused by lymphoma cells building up in the lymph nodes, which makes them bigger. Other symptoms may include loss of appetite and tiredness (fatigue).

B symptoms

Rarely, some people have:

  • drenching night sweats
  • high temperatures (fevers) with no obvious cause
  • unexplained weight loss.

These are called B symptoms.


Diagnosing NMZL

The most common test for NMZL 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.

Doctors may also want to look inside the stomach with a long, flexible tube called an endoscope. 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.

Other tests may include:

  • blood tests
  • x-rays and scans
  • bone marrow samples.

Doctors use the information from all these tests to find out more about the lymphoma, such as its stage and grade.


Staging and grading of NMZL

Staging

The stage of the lymphoma describes which areas of the body are affected by lymphoma. This information helps doctors plan the right treatment for you.

The stage of a lymphoma is usually described using numbers from 1 to 4. Stages 1 and 2 are also called early-stage, limited or localised lymphoma. Stages 3 and 4 are also called advanced lymphoma.

As well as giving each stage a number, doctors often add the letters A or B. A means you do not have B symptoms. B means you do have B symptoms.

Sometimes the lymphoma can affect areas outside the lymph nodes. This is called extranodal lymphoma, and the stage will include the letter E (for extranodal).

Grading

Non-Hodgkin lymphomas are divided into two groups:

  • Low-grade (indolent) lymphomas usually, which grow slowly.
  • High-grade (aggressive) lymphomas, which grow more quickly.

NMZL is a low-grade lymphoma and usually develops slowly. Sometimes it can change (transform) to become a high-grade lymphoma.


Treating NMZL

NMZL often develops slowly. Many people may not need treatment straight away, or even for months or years. If you don’t need immediate treatment, you will have regular check-ups at the hospital. Your lymphoma doctor will examine you and do blood tests. This is called watch and wait

Treatment depends on:

  • the stage of the lymphoma
  • whether you have symptoms that are causing problems.

The most commonly used treatment is a combination of chemotherapy and a targeted therapy called rituximab. Most people have their treatment as an outpatient.

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.

If NMZL comes back after treatment, further treatment can often be successful at getting the lymphoma into remission again.

Targeted therapy

A targeted therapy called rituximab is commonly used to treat NMZL. It works by targeting proteins on the surface of B-cells. This makes the body destroy these cells.

Rituximab is given as a drip into a vein. It can be given on its own, but it is usually given with chemotherapy. Some people who have a good response to chemotherapy and rituximab may then have rituximab on its own for up to 2 years. This is known as maintenance treatment.

Chemotherapy

Chemotherapy is the use of anti-cancer (cytotoxic) drugs to destroy cancer cells.

It is an important treatment for NMZL and can often get the lymphoma into remission. It is usually given in combination with rituximab.

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

Chemotherapy treatments for NMZL include the following:

  • R-CVP – this is made up of rituximab, the chemotherapy drugs cyclophosphamide and vincristine, and prednisolone (a steroid). It is given as a drip into a vein, usually once every three weeks.
  • R-CHOP – this is made up of made up of rituximab, the chemotherapy drugs cyclophosphamide, doxorubicin (hydroxydaunomycin) and vincristine (Oncovin®), and prednisolone.
  • 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.

Steroid therapy

Steroids are drugs that are often given with chemotherapy to help treat lymphomas. They also help you feel better and can make you feel less sick.

Prednisolone tablets are the most commonly used steroid to treat NMZL.

Stem cell treatment (transplants)

This treatment is sometimes used to treat lymphoma that has come back after treatment. It is an intensive treatment, so it is not suitable for everyone.

Stem cells are a type of blood cell that can make all other types of blood cells. There are two different types of stem cell treatment:

High-dose treatment with stem cell support (autologous stem cell transplant)

Some people have treatment to put the lymphoma into remission. Then some of their own stem cells are collected from their blood and stored. Then they have high doses of chemotherapy to try to destroy any remaining lymphoma cells. After this, their stem cells are returned through a drip (like a blood transfusion). The stem cells help their blood cell numbers recover from the effects of chemotherapy.

Donor transplant (allogeneic transplant)

Some people have chemotherapy to put the lymphoma into remission and are then given stem cells from another person (a donor).

Radiotherapy

Radiotherapy uses high-energy rays to destroy cancer cells, while causing as little harm as possible to the 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.

Clinical trials

Your lymphoma doctor may talk to you about having treatment as part of a clinical trial. Clinical trials test new treatments or new ways of giving treatments.


Follow-up after treatment

After treatment, you will have regular check-ups. 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. We have more information about follow-up.


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. You can also call our cancer support specialists free on 0808 808 00 00. The organisations below also offer information and support:

  • Bloodwise offers support and information to people affected by blood cancers, including lymphoma.
  • Lymphoma Action 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.