Mantle cell lymphoma

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

The lymphatic system has tubes that branch through all parts of the body. It carries a colourless liquid called lymph. This liquid 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
• T cells

Mantle cell lymphoma affects the B cells. It develops in the part of the lymph node called the mantle zone. 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

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

Painless swellings

The most common symptom of NHL is one or more painless swellings in the:

• neck
• armpit
• groin

These swellings are enlarged lymph nodes.

General symptoms (B symptoms)

You might 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)
• unexplained itching

Doctors call this group of symptoms B symptoms. Some people with NHL have these symptoms, but many don't.

Other symptoms

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, tummy pain and sickness. 

Stages and grades

Doctors put NHLs into 2 groups, depending on how quickly they are likely to grow and spread. The 2 groups are:

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

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

Doctors use your stage to decide on the best treatment. Your stage means the number and position of lymph nodes or other organs affected by 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

You might have radiotherapy as a treatment on its own if you have stage 1 or 2 mantle cell lymphoma.

You might have radiotherapy and chemotherapy together (chemoradiotherapy) for advanced stages of the disease.

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

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