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Mantle cell lymphoma

Mantle cell lymphoma is a rare type of non Hodgkin lymphoma (NHL). Only about 5 out of every 100 people (5%) diagnosed with NHL have mantle cell lymphoma. It mainly affects men who are over 50. The symptoms of mantle cell lymphoma are similar to those of most other types of NHL.

The grade of an NHL (the appearance of the cells under the microscope) helps doctors to decide on the most appropriate treatment. Mantle cell lymphoma looks like a low grade lymphoma under the microscope. But it often behaves aggressively, like a high grade lymphoma. Unfortunately it is often widespread when it is diagnosed, involving lymph nodes, bone marrow, and, very often, the spleen.

Treating mantle cell lymphoma

Treatment for mantle cell lymphoma can be similar to treatment for other types of NHL. Unfortunately mantle cell lymphoma is difficult to cure. It is an aggressive type of NHL and most people are diagnosed in the later stages of the disease. Aggressive means that the lymphoma grows very quickly. Mantle cell lymphoma often comes back relatively soon after you get it into remission

Chemotherapy is the most common treatment for mantle cell lymphoma. Standard chemotherapy treatments for mantle cell lymphoma are changing following the results of clinical trials that looked at drugs such as rituximab and cytarabine.
 

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What mantle cell lymphoma is

Mantle cell lymphoma is a rare type of non Hodgkin lymphoma (NHL). Only about 5 to 10 out of every 100 people (5 to 10%) diagnosed with NHL have mantle cell lymphoma. It mainly affects men who are over 50.

Non Hodgkin lymphoma is cancer of the lymphatic system. The lymphatic system has tubes that branch through all parts of the body, similar to the arteries and veins that carry blood in the circulatory system. The lymphatic system carries a colourless liquid called lymph. This fluid circulates around the body tissues. It contains a high number of white blood cells (lymphocytes), which fight infection.

When you have lymphoma some of your white blood cells (lymphocytes) don't work properly. They start to divide constantly but don't develop fully. So they can't fight infection as normal white blood cells do. There are two main types of lymphocytes – B cells and T cells. Mantle cell lymphoma affects the B cells. The abnormal B lymphocytes start to collect in the lymph nodes or body organs. They can then form tumours and begin to cause problems within the lymphatic system or the organ where they are growing.

 

Symptoms of mantle cell lymphoma

The symptoms of mantle cell lymphoma are similar to those of most other types of NHL. The most common symptom is one or more painless swellings in the

  • Neck
  • Armpit
  • Groin

Each swelling is an enlarged lymph node. If you have a swollen lymph node that does not go away after 6 weeks, you should see your GP.

You may have other general symptoms such as

  • Heavy sweating at night
  • Temperatures that come and go with no obvious cause
  • Losing a lot of weight (more than one tenth of your total weight)

Doctors call this group of symptoms B symptoms. Some people with non Hodgkin lymphoma have them, but most don't. Mantle cell lymphoma can spread to the bowel and in rare cases to the stomach. If this happens, it can cause symptoms such as diarrhoea, abdominal (tummy) pain and sickness.

It is important for you to tell your doctor about any symptoms like this. Knowing that you have these symptoms helps your doctor to know which type of non Hodgkin lymphoma you have. Then they know which type of treatment you need. There is more information about the symptoms of NHL in this section.

 

Stage and grade of mantle cell lymphoma

Doctors put non Hodgkin lymphomas into 2 groups.  The groups depend on how quickly they are likely to grow and spread

  • Low grade or indolent (slow growing)
  • High grade or aggressive (fast growing)

The appearance of the cells under the microscope (grade of NHL) helps doctors to decide on the best treatment for you. Mantle cell lymphoma looks like a low grade lymphoma under the microscope. But it often behaves aggressively, more like a high grade lymphoma. Unfortunately it is often widespread when it is diagnosed. This means it may involve lymph nodes, the bone marrow and the spleen.

The stage is also very important in deciding about treatment. The stage of NHL means the number and position of the lymph nodes or other affected organs. There is detailed information about the stages of NHL in this section.

 

Treating mantle cell lymphoma

Treatment for mantle cell lymphoma can be similar to treatment for other types of NHL. But it is difficult to cure. It is an aggressive type of NHL and people are often diagnosed in the later stages of the disease. By aggressive, we mean that the lymphoma grows very quickly. Unfortunately, it often comes back fairly soon after you get it into remission. If you have mantle cell lymphoma your doctor may suggest that you join a clinical trial. Trials most often compare the best known standard treatment with an experimental treatment that doctors hope will work better.

Doctors treat mantle cell lymphoma with one or more of these treatments

Chemotherapy

Chemotherapy is the most common type of treatment for people with mantle cell lymphoma. Standard chemotherapy treatments are changing following the results of clinical trials that looked at drugs such as cytarabine and rituximab. For people who are not fit enough to have very aggressive chemotherapy, the most common chemotherapy drugs used are

Click on the links to find out the specific side effects of these drugs. You are likely to have a type of biological therapy called rituximab with your chemotherapy.

If you are fit and under 65 years old you may have a combination of rituximab and CHOP chemotherapy (R-CHOP) alternating with rituximab and high dose cytarabine. You may then have a transplant.

Transplant

If you are young and fit enough, you may have high dose BEAM chemotherapy followed by a transplant using your own stem cells. You may also have a bone marrow or stem cell transplant if your mantle cell lymphoma has come back after chemotherapy. High dose chemotherapy increases the risk of complications, such as infection, but gives the best chance of curing the lymphoma. You need to stay in hospital for a few weeks to have this treatment. It is hard going but you will get a lot of support from the staff during your stay in hospital. Look at the section about transplants for more information.

A trial is looking at having a stem cell transplant using lower doses of chemotherapy in combination with alemtuzumab (MabCampath) as a first treatment for mantle cell lymphoma. This is called reduced intensity conditioning (RIC), mini transplant or mini allogeneic transplant (mini allo). The trial aims to find out if a mini allo stem cell transplant is a safe treatment for people with mantle cell lymphoma. It also aims to find out if it is better to have a transplant when people first go into remission, rather than waiting until the lymphoma comes back. You can find out about the mini allo transplant trial on our clinical trials database.

Radiotherapy

If you have stage 1 or 2 mantle cell lymphoma, your doctor may treat you with radiotherapy. Or you may have radiotherapy and chemotherapy together for advanced stages of the disease. There is detailed information about radiotherapy in this section.

Steroid therapy

Steroids are substances made naturally in the body. They can also be made artificially and used as drugs. Steroids are used for many different reasons, and for many different illnesses and conditions. In non Hodgkin lymphoma, you take steroids along with your chemotherapy drugs. Doctors have found that the treatment is more successful when you have steroids and chemotherapy together. The common steroid drugs include

  • Prednisolone
  • Dexamethasone
  • Methylprednisolone

There is more information about steroid therapy in the treating non Hodgkin lymphoma section.

Biological therapy

There are many different types of biological therapy. They act on processes in cancer cells. For example they can change the way cells signal to each other. Or they can stimulate the body to attack or control the growth of cancer cells. One of these drugs is rituximab. It is a type of monoclonal antibody. You may have it with chemotherapy

Another type of biological therapy is bortezomib. It is a proteasome inhibitor. This means that it interferes with the chemicals inside cells, making proteins build up and kill the cells. Cancer cells are more sensitive to proteasome inhibitors than normal cells. There is a trial looking at CHOP chemotherapy with or without bortezomib for relapsed mantle cell lymphoma. Researchers want to find out if this works better than just the chemotherapy on its own. Other biological therapies being looked at for treating mantle cell lymphoma include lenalidomide (Revlimid), temsirolimus, ibrutinib and olaparib.

Look at the treating non Hodgkin lymphoma section for more information about treatment with biological therapies. There is also information about new monoclonal antibody treatments for NHL in the non Hodgkin lymphoma research section.

 

Research into mantle cell lymphoma

You can find out more about research into treatment for mantle cell lymphoma on our clinical trials database. Type 'mantle cell lymphoma' into the search box. If you want to see all the trials, tick the boxes for closed trials and trial results.

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Updated: 6 November 2012