Mantle cell lymphoma

Mantle cell lymphoma is a rare type of non Hodgkin's lymphoma (NHL). There are about 20 different types of 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’s 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.

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's lymphoma have them, but most don't. Mantle cell lymphoma can spread to the bowel and stomach. If this happens, it can cause symptoms such as diarrhoea 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's lymphoma you have. Then they know which type of treatment you need. There is more information about the symptoms of NHL in this section of CancerHelp UK.

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

• Low grade or slow growing
• High grade or 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 of CancerHelp UK.

Treatment for mantle cell lymphoma is 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
• Transplant
• Radiotherapy
• Steroid therapy
• Biological therapy

Chemotherapy

Chemotherapy is the most common type of treatment for people with mantle cell lymphoma. For people who are not fit enough to have very aggressive chemotherapy, the most common drugs used are

• Chlorambucil
• Cyclophosphamide, vincristine, and the steroid drug prednisolone (CVP)
• Cyclophosphamide, doxorubicin, vincristine, and prednisolone (CHOP)

Click on the links to find out the specific side effects of these drugs. Mantle cell lymphoma may also be treated with a combination of chemotherapy and rituximab.

Transplant

If mantle cell lymphoma comes back, some people have high dose chemotherapy with a bone marrow or stem cell transplant. 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 (Campath) 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 the non Hodgkin’s lymphoma section of CancerHelp UK.

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's 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’s lymphoma section of CancerHelp UK.

Biological therapy

Biological therapies are treatments that use natural substances from the body (or drugs developed from them) or that change the way cells signal to each other. They can stimulate the body to attack or control the growth of cancer cells. One of these drugs is bortezomib (Velcade). Bortezomib 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.

Rituximab is another type of biological therapy used to treat mantle cell lymphoma. Rituximab is a type of monoclonal antibody. You may have it with chemotherapy. Other newer monoclonal antibodies are being researched for treating mantle cell lymphoma. Look at the treating non Hodgkin’s 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's lymphoma research section.

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.