Mantle cell lymphoma

Mantle cell lymphoma is a type of non-Hodgkin lymphoma (NHL). It develops when the body makes abnormal B-lymphocytes – the lymphoma cells. (B-lymphocytes are white blood cells that fight infection.) The lymphoma cells build up in lymph nodes. 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 and 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 elsewhere. This is called staging.

MCL is usually treated with a combination of chemotherapy drugs and a monoclonal antibody called rituximab. Other treatments that may be used include 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 haematology doctor.

Mantle cell lymphoma

Mantle cell lymphoma is a 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 made up of organs such as the bone marrow, thymus, spleen, and the lymph nodes (or lymph glands). 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 that the body doesn't need. This includes bacteria, viruses, damaged cells and cancer cells.

The lymphatic system
The lymphatic system

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

Lymphocytes start to grow in the bone marrow, where blood cells are made. The two main types of lymphocytes 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 where either T-cells or B-cells grow in an uncontrolled way. 

There are many different types of NHL. The types are grouped (or classified) according to certain characteristics, such as the type of cell involved (B-cell or T-cell). The most widely used classification system is produced by the World Health Organisation.


What is mantle cell lymphoma?

Mantle cell lymphoma (also called MCL) is a rare type of B-cell lymphoma. It is mainly diagnosed in people over the age of 60.


Causes of mantle cell lymphoma

The causes of mantle cell lymphoma are unknown. It is not infectious and cannot be passed on to other people.


Signs and symptoms of mantle cell lymphoma

The first sign is often a painless swelling in the neck, armpit or groin, caused by enlarged lymph nodes. Usually, more than one group of nodes is affected. Other areas of the body may also be affected, such as the bone marrow, bowel, stomach, liver or spleen.

Other symptoms may include loss of appetite and tiredness (fatigue).

Some people have night sweats, high temperatures (fevers) and weight loss. These are known as B symptoms. If the lymphoma affects the bowel or stomach, it may cause diarrhoea and sickness (nausea).


How mantle cell lymphoma is diagnosed

A diagnosis is made by removing an enlarged lymph node (a biopsy) and examining the cells under a microscope. This is a small operation and may be done under local or general anaesthetic. Biopsies may also be taken from other body tissues.

Additional tests include:

  • blood tests
  • x-rays and scans
  • a lumbar puncture to examine the cerebrospinal fluid (which protects your brain and spinal cord)
  • bone marrow samples.

The results of these tests are used to find out more about the lymphoma and how far it has spread in the body. This information is used to help decide which treatment is most appropriate. You can read more about these tests in our information on tests for NHL.


Staging and grading of mantle cell lymphoma

Staging

The stage of NHL 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.

There are four stages:

Stage 1

One group of lymph nodes is affected.

Stage 2

Two or more groups of lymph nodes are affected, and all the affected lymph nodes are either above or below the diaphragm (a sheet of muscle under the lungs).

Stage 3

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

Stage 4

The lymphoma has spread beyond the lymph nodes to other organs such as the bones, liver or lungs.

Mantle cell lymphoma is usually diagnosed at stage 3 or stage 4.

As well as giving each stage a number, doctors also use either the letter A or B to show whether or not 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. B symptoms are often present with mantle cell lymphoma.

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

Grading

For practical purposes, 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.

Mantle cell lymphoma is usually treated as an aggressive lymphoma.


Treatment for mantle cell lymphoma

Chemotherapy in combination with a monoclonal antibody drug called rituximab (Mabthera ®) is the main treatment for mantle cell lymphoma. Some people may also have stem cell 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.

Other treatments that may be used include steroids or radiotherapy. You may have a combination of treatments.

Mantle cell lymphoma 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.

Monoclonal antibody therapy

Monoclonal antibodies are drugs that recognise, target and stick to particular proteins on the surface of cancer cells, and can stimulate the body’s immune system to destroy these cells. Rituximab is the monoclonal antibody commonly used to treat MCL. It's given as a drip into a vein (intravenous infusion) and is usually given with chemotherapy. Some people who have a good response to treatment may continue to be given regular rituximab infusions for up to two years. This is known as maintenance treatment. Other monoclonal antibodies may be given, usually as part of a cancer research trial.

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 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:

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. These treatments may be given before high-dose chemotherapy with stem cell treatment.

  • R-HCVAD (rituximab, cytarabine, cyclophosphamide, vincristine, doxorubicin and the steroid dexamethasone)
  • R_Maxi CHOP/H Ara-C (rituximab, cyclophosphamide, vincristine, doxorubicin, cytarabine and the steroid prednisolone).

Steroid therapy

Steroids are often given with chemotherapy to help 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 a special type of blood cell that can make all other types of blood cells.

This is an intensive treatment and is only suitable for people who are fit enough to cope with the side effects.

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 treatment with stem cell support.

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

Radiotherapy

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

It may be used when the lymphoma cells are contained in one or two groups of lymph nodes in the same part of the body (stage 1 or 2).

It may also sometimes be used to relieve symptoms such as pain in a particular area.

Newer treatments

Bortezomib (Velcade ®)

Bortezomib blocks signals in cells that are involved in cell growth. This may cause the lymphoma cells to die. It is given as an injection into a vein and may be used when other treatments have been tried.

Temsirolimus

Temsirolimus interferes with signals that tell cells to grow and divide. Temsirolimus is known as an mTOR inhibitor. It's 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's taken as a tablet.


Clinical trials

New treatments for mantle cell 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, which 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.


Information and support

Everyone has their own way of dealing with their 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 contact our cancer support specialists or the organisations below for more information and support.


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.