Mantle cell lymphoma

Mantle cell lymphoma (MCL) is a type of non-Hodgkin lymphoma (NHL). It develops when the body makes abnormal B-cells – the lymphoma cells. B-cells are white blood cells that fight infection. The lymphoma cells build up in lymph nodes, which makes them bigger. Sometimes lymphoma cells begin in other parts of the body. This is called extranodal disease.

The most common symptom is a painless swelling in the neck, armpit or groin. Other symptoms may include:

  • tiredness
  • weight loss
  • night sweats
  • high temperatures (fevers).

To diagnose MCL, 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. This is called staging.

MCL is usually treated with a combination of chemotherapy drugs and a targeted therapy called rituximab. Some people may also have stem cell treatment. Other treatments include different targeted therapy drugs and radiotherapy.

You may be invited to join a clinical trial looking at new ways of treating MCL. You can talk about this with your lymphoma doctor.

What is mantle cell lymphoma (MCL)?

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.

Mantle cell lymphoma (MCL) is a 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.


Causes and risk factors for MCL

The causes of mantle cell lymphoma are not known. It mainly occurs in people over the age of 60 and is more common in men than women. Like other cancers, it is not infectious and cannot be passed on to other people.

We have more information about risk factors for lymphoma.


Signs and symptoms of MCL

Often the first sign of MCL is a painless swelling in the neck, armpit or groin. This is caused by abnormal B-cells building up in the lymph nodes, which makes them bigger. Other areas of the body may also be affected, such as the:

  • bone marrow
  • bowel
  • stomach
  • liver
  • spleen.

Other symptoms may include loss of appetite and tiredness (fatigue). If the lymphoma affects the bowel or stomach, it may cause diarrhoea and sickness (nausea).

Sometimes lymphoma cells may build up in the bone marrow, where blood cells are made. This can cause lower than normal numbers of healthy blood cells in the blood. If the number of red blood cells in your blood is too low, you may be pale, tired and get breathless easily. This is called anaemia. If the number of blood-clotting cells (platelets) in your blood is too low, you may bruise or bleed easily.

B symptoms

Some people also have:

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

These are called B symptoms.


Diagnosing 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. You may also have biopsies taken from other areas of the body.

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 MCL

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.

MCL is usually diagnosed at stage 3 or stage 4.

As well as giving each stage a number, doctors also 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, which usually grow slowly.
  • High-grade (aggressive) lymphomas, which grow more quickly.

MCL is usually treated as a high-grade lymphoma.


Treating MCL

The main treatment for MCL is chemotherapy in combination with a targeted therapy drug called rituximab. Some people may also have stem cell treatment. Radiotherapy and other targeted treatments are sometimes used. You may have a combination of treatments.

The aim of treatment is to get rid of as much of the lymphoma as possible. If there are no signs of MCL after treatment, this is called a complete remission.

MCL usually comes back after treatment. Doctors are trying to find improved ways of treating it and controlling it for longer periods. Treatments may be given as part of a clinical research trial.

Rituximab

Rituximab is a is a type of treatment called a targeted therapy. It targets proteins on the surface of B-cells. This makes the body destroy these cells.

Rituximab is given as a drip into a vein (infusion). It is usually given with chemotherapy. It may also be given with another targeted therapy called bortezomib.

If the MCL responds well to treatment, you may continue to have regular rituximab treatments. The aim is to keep the lymphoma in remission. This is called maintenance treatment.

Your lymphoma doctor can tell you how long maintenance treatment might continue for.

Chemotherapy

Chemotherapy is the use of anti-cancer (cytotoxic) drugs to destroy cancer cells. It can be given into a vein (intravenously) or as tablets. It is usually given in combination with rituximab.

There are several chemotherapy drugs that can be used to treat MCL. The chemotherapy drugs you have will depend on your general fitness and how the side effects of chemotherapy may affect you.

Combinations of rituximab and chemotherapy that may be used include the following:

  • R-chlorambucil – this is made up of rituximab and chlorambucil.
  • R-bendamustine – this is made up of rituximab and bendamustine.
  • 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.
  • R-CHOP – this is made up of rituximab, prednisolone, and the chemotherapy drugs vincristine, cyclophosphamide and doxorubicin (hydroxydaunomycin). These are given as a drip into a vein.

People who are fit enough to cope with the side effects of intensive treatments may be given treatment that includes high doses of the chemotherapy drug cytarabine. The following treatments may be given before high-dose chemotherapy with stem cell treatment:

  • R-Hyper-CVAD – this is made up of rituximab, cytarabine, cyclophosphamide, vincristine, doxorubicin (Adriamycin), methotrexate and the steroid dexamethasone.
  • R-Maxi CHOP/H Ara-C – this is made up of rituximab, cyclophosphamide, hydroxy-doxorubicin, vincristine (oncovin), the steroid prednisolone and high dose cytarabine (Ara-C).

Steroids

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

High-dose chemotherapy with stem cell support

This is an intensive treatment. It is only suitable for people who are fit enough to cope with the side effects. The aim is to put the lymphoma into remission for as long as possible.

Stem cells are a type of blood cell that can make all other types of blood cells. You have chemotherapy and rituximab to put the lymphoma into remission. Then some of your stem cells are collected from your blood and stored. After this you are given high doses of chemotherapy to destroy any remaining lymphoma cells. Your stem cells are then returned to you through a drip (like a blood transfusion). The stem cells help your blood cells recover from the effects of chemotherapy.

Radiotherapy

Radiotherapy uses high-energy rays to destroy cancer cells, while doing as little harm as possible to nearby healthy cells.

It may be used:

  • if the lymphoma is contained in 1 or 2 groups of lymph nodes in the same part of the body (stage 1 or 2)
  • to relieve symptoms, such as pain, in a particular area.

Other targeted therapies

Targeted therapies interfere with the way cancer cells divide and grow. This can shrink the cancer or stop it growing.

The targeted therapies rituximab and bortezomib can be used as first treatments for MCL. Other targeted therapies may be used to treat MCL that has come back after treatment (relapsed). They may also be given to treat newly diagnosed MCL as part of a clinical trial. They are usually given in combination with chemotherapy and rituximab.

Bortezomib (Velcade®)

Bortezomib blocks signals in cancer cells that are involved in cell growth. It may be given in combination with rituximab and chemotherapy to treat people who are not fit enough to have stem cell treatment. Bortezomib may also be used to treat MCL that has come back after treatment.

It is given as an injection into a vein or as an injection under the skin (subcutaneously).

Temsirolimus

Temsirolimus interferes with signals that tell cells to grow and divide. It is given into a vein as a drip (infusion).

Lenalidomide (Revlimid®)

Lenalidomide blocks the development of new blood vessels. Cancer cells need to make new blood vessels so they can grow and spread. It is taken as a tablet.

Ibrutinib (Imbruvica®)

Ibrutinib blocks a protein that helps the lymphoma cells grow. It is taken as a tablet every day.


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.
  • 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.