Mantle cell lymphoma
This information is about a type of non-Hodgkin lymphoma (NHL) called mantle cell lymphoma.
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 (NHL) is a cancer of the lymphatic system. The lymphatic system is part of the body’s immune system and helps us fight infection. It's 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.
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. The most widely used classification system is produced by the World Health Organisation.
Mantle cell is a rare type of lymphoma. It is a cancer of the B-cells. People with mantle cell lymphoma are usually aged 60-65, and it's more common in men than in women.
Causes of mantle cell lymphoma
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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
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The first sign is often a painless swelling in the neck, armpit or groin that is 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 can cause symptoms such as diarrhoea and sickness (nausea).
How mantle cell lymphoma is diagnosed
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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:
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 about tests for non-Hodgkin lymphoma.
Staging and grading of mantle cell lymphoma
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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:
One group of lymph nodes is affected.
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 lung).
The lymphoma is in lymph nodes both above and below the diaphragm.
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).
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.
When the cells are examined under a microscope, mantle cell lymphoma looks like an indolent lymphoma. But it usually behaves more like an aggressive lymphoma.
Treatment for mantle cell lymphoma
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Chemotherapy is the use of anti-cancer (cytotoxic) drugs to destroy cancer cells, and is the main treatment for mantle cell lymphoma. Some people may also have stem cell treatments.
Other treatments are also used, such as monoclonal antibodies, steroids and radiotherapy. You may have a combination of different treatments.
Other newer biological treatments, such as drugs called temsirolimus and lenalidomide, may be given after other treatments have been tried.
Mantle cell lymphoma can come back after it is first treated. Doctors are trying to find improved ways of treating it and controlling it for longer periods. Many of the treatments are given in a research trial.
The chemotherapy you have will depend on the stage of the lymphoma and how the side effects of chemotherapy are likely to affect you.
Chemotherapy can be given as a drip (infusion), into a vein (intravenously), and also as tablets. It is often given in combination with a monoclonal antibody drug called rituximab.
Some of the following chemotherapy treatments are used:
R-CHOP – vincristine, doxorubicin, cyclophosphamide, the steroid prednisolone and the monoclonal antibody rituximab.
FC-R – fludarabine (Fludara®), cyclophosphamide and rituximab.
FCM-R – fludarabine (Fludara®), cyclophosphamide, mitoxantrone and rituximab.
R-HCVAD – cytarabine, cyclophosphamide, doxorubicin, vincristine, the steroid dexamethasone and rituximab.
R_Maxi CHOP/H Ara-C – vincristine, doxorubicin, cyclophosphamide, cytarabine, the steroid prednisolone and rituximab.
Younger people who are better able to cope with the side effects of very intensive treatments may be given treatment that includes high-dose cytarabine, such as R-HCVD or RMaxi CHOP/ H-araC. These treatments can be given on their own or before high-dose chemotherapy with stem cell treatment.
If you aren't well enough to cope with the side effects of a combination of chemotherapy drugs, you may be given a single chemotherapy drug. Chemotherapy drugs that may be used as single drug treatments include bendamustine, chlorambucil, cyclophosphamide and fludarabine. These chemotherapy drugs may also be given with rituximab.
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 main monoclonal antibody used to treat mantle cell lymphoma. It's given as a drip into a vein (intravenous infusion). Rituximab is usually given with chemotherapy.
Some people who have a good response to chemotherapy may be given follow-up treatment with regular rituximab infusions for up to two years. This is known as maintenance treatment.
Radioimmunotherapy combines a monoclonal antibody drug and radiation therapy to kill cancer cells, such as 90Y-ibritumomab tiuxetan (Zevalin®).
Other monoclonal antibodies may also be given, usually as part of a cancer research trial.
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 treatment is not suitable for everyone and is not done routinely. Doctors take into account a person's general health and fitness 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 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 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).
Steroids are drugs that are often given with chemotherapy to help treat lymphomas. They also help you feel better and can reduce feelings of sickness.
Temsirolimus is a drug that may be given when mantle cell lymphoma has not responded to other treatments or has come back. It works by interfering with the 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 is a drug that affects the way the immune system works. It may be used when other treatments have already been tried, usually in a research trial. It's taken as a tablet.
Bortezomib is a type of treatment called a proteasome inhibitor. It works by blocking 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.
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
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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 listed here for more information and support.
Other useful organisations
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Leukaemia CARE is a national group promoting the welfare of people with leukaemia and related blood disorders, including NHL. It has regional support groups in many counties.
The Lymphoma Association
The Lymphoma Association
gives emotional support, advice and information on all aspects of NHL. It has a national network of people with lymphoma and local groups.
This section has been compiled using information from a number of reliable sources, including:
Facilities for the treatment of adults with haematological malignancies. Levels of Care. British Committee for Standards in Haematology. 2010.
Lee, et al. Wintrobe’s Clinical Haematology. 12th edition. 2009. Lippincott Williams and Wilkins.
McKay P, et al. Guidelines for the investigation and management of mantle cell lymphoma. BSCH guidelines. 2012.,
Parker A, et al. Best Practice in Lymphoma Diagnosis and Reporting. Specific disease appendix. BSCH guidelines. 2010.
Thank you to Professor Rajnish Gupta, Consultant Medical Oncologist, and the people affected by cancer who reviewed this edition. Reviewing information is just one of the ways you could help when you join our Cancer Voices network.