Mantle cell lymphoma

Mantle cell lymphoma is a rare type of B cell non-Hodgkin lymphoma (NHL). 

NHL is a type of blood cancer that affects white blood cells called lymphocytes . It is a cancer of the lymphatic system . 

Treatment usually includes chemotherapy together with a targeted drug called rituximab. Doctors call this chemoimmunotherapy.

What is mantle cell lymphoma?

Mantle cell lymphoma is a rare type of B cell non-Hodgkin lymphoma (NHL).

Lymphoma develops when white blood cells called lymphocytes grow out of control. They move around your body in your blood and your lymphatic system. 

The lymphatic system is an important part of our immune system. It has tubes that branch through all parts of the body.

These tubes are called lymph vessels or lymphatic vessels and they carry a straw coloured liquid called lymph. This liquid circulates around the body tissues. It contains a high number of white blood cells (lymphocytes) which fight infection.

There are two types of lymphocytes: T lymphocytes (T cells) and B lymphocytes (B cells). 

Mantle cell lymphoma develops when the body makes abnormal B lymphocytes. So it is a type of B cell lymphoma. It develops in the part of the lymph node called the mantle zone.

The abnormal lymphocytes build up in the lymph nodes or in other body organs. They don't work properly. So they can't fight infection as normal white blood cells do. They form tumours and begin to cause problems within the lymphatic system or the organ where they are growing.

Symptoms

The symptoms of mantle cell lymphoma are similar to those of most other types of NHL.

Painless swellings

The most common symptom of non-Hodgkin lymphoma (NHL) is one or more painless swellings in the:

• neck

• armpit

• groin

These swellings are enlarged lymph nodes.

General symptoms (B symptoms)

Other symptoms

Mantle cell lymphoma can develop outside the lymph nodes. The symptoms you have depend on where it grows. For example, it can grow in your:

• tummy (abdomen) or bowel - this might cause pain, diarrhoea or sickness
• bone marrow  - this can cause anaemia , bruising or bleeding problems
• spleen - this can make you feel full very quickly when you eat, or cause discomfort behind your ribs

Stages and grades of mantle cell lymphoma

Grade

Grade describes how the cells look under a microscope. Your grade tells the doctor how quickly the lymphoma is likely to grow and spread. Doctors put NHL into 2 grades:

• low grade (slow growing)
• high grade (fast growing)

Mantle cell lymphoma looks like a low grade lymphoma under the microscope. But it often grows faster, more like a high grade lymphoma.

Stage

You have tests to find out the number of places in your body affected by lymphoma, and where these are. This is your stage. Doctors use your stage to plan your treatment.

There are 4 main stages of NHL (stage 1 to stage 4). Or your doctors might talk about early (limited) stage and advanced stage lymphoma. 

Early stage usually means either stage 1 or stage 2 lymphoma. Advanced stage usually means either stage 3 or stage 4 lymphoma. 

Mantle cell lymphoma can grow quickly. So unfortunately it's often widespread at diagnosis. This means you may have lymphoma cells in your lymph nodes, bone marrow and spleen. 

Treatment

Treatment for mantle cell lymphoma can be similar to treatment for other types of NHL. 

It grows quickly and is often diagnosed when it is in a later stage. Treatment can sometimes get rid of the lymphoma completely. But unfortunately it can come back fairly soon afterwards.

Chemotherapy and immunotherapy

You usually have some chemotherapy drugs in combination with immunotherapy and steroids. Chemotherapy aims to damage and kill cancer cells as they divide. Immunotherapy uses the immune system to fight cancer. 

The most commonly used combinations are:

• R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and dexamethasone)
• R-DHAP (rituximab, cytarabine, cisplatin and dexamethasone)
• Nordic protocol (rituximab, cyclophosphamide, doxorubicin, vincristine, cytarabine, prednisolone)
• Bendamustine and rituximab

You might also have the drug lenalidomide.

Steroids

Steroids are substances made naturally in the body. They can also be made artificially and used as drugs.

For NHL, you take steroids along with your chemotherapy drugs. The treatment is more successful when you have steroids and chemotherapy together. Commonly used steroid drugs include prednisolone, dexamethasone and methylprednisolone.

Radiotherapy

Stem cell transplant

You might have a stem cell treatment if you are fit enough for high dose treatment  and your lymphoma has responded to chemotherapy treatment. You may also have this treatment if your mantle cell lymphoma has come back after previous treatments.

There are different combinations of high dose treatment. The most common include:

• BEAM (carmustine, etoposide, cytarabine and melphalan)
• LEAM (lomustine, etoposide, cytarabine and melphalan)

After high dose treatment you have a transplant using your own stem cells. 

You need to stay in hospital for a couple of weeks to have these treatments. It is hard going, but you get a lot of support from the staff during your stay in hospital and afterwards.

Less intensive treatment

If you are unable to have intensive treatment you might have:

• bortezomib (a targeted cancer drug) 

• chlorambucil (a chemotherapy drug) 

• ibrutinib

Research

Your doctor might ask if you’d like to take part in a clinical trial. Doctors and researchers do trials to:

• make existing treatments better

• develop new treatments

Treatments being tested for mantle cell lymphoma include:

• olaparib

• low intensity (mini) transplants

Coping with NHL

It can be difficult finding our you have NHL. Some people find it can be particularly worrying in the run up to check up appointments.

Advice and support is available to help you cope with NHL and its treatment.

Our discussion forum Cancer Chat is a place for anyone affected by cancer. You can share experiences, stories and information with other people who know what you are going through.