Mantle cell lymphoma is a rare type of B cell non Hodgkin lymphoma (NHL). Find out about the symptoms and treatment.
What lymphoma is
Non Hodgkin lymphoma (NHL) is a 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. 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 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.
How common is it
Mantle cell lymphoma is rare. 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.
The symptoms of mantle cell lymphoma are similar to those of most other types of NHL.
The most common symptom of non Hodgkin lymphoma is one or more painless swellings in the:
Each swelling is an enlarged lymph node.
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)
Doctors call this group of symptoms B symptoms. Some people may also have unexplained itching.
Some people with NHL have these symptoms, but many 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, 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 for mantle cell lymphoma can be similar to treatment for other types of NHL.
But unfortunately, mantle cell lymphoma can be difficult to cure. It grows quickly and is often diagnosed when it is in a later stage. Treatment can sometimes get rid of the lymphoma completely. But it often comes back fairly soon afterwards.
Chemotherapy is the most common treatment for people with mantle cell lymphoma. The most commonly used chemotherapy drugs are:
- cyclophosphamide, doxorubicin, vincristine, and prednisolone (CHOP), sometimes combined with the biological therapy drug rituximab (Mabthera) to make R-CHOP
- fludarabine and cyclophosphamide
- cyclophosphamide, vincristine and prednisolone (CVP combination chemotherapy)
Stem cell or bone marrow transplant
You might have this treatment if you are fit enough and under 65 years old. You may also have it if your mantle cell lymphoma has come back after previous chemotherapy. High dose chemotherapy increases the risk of complications such as infection. But it gives the best chance of curing the lymphoma.
You might have:
- R-CHOP alternating with the biological therapy drug rituximab (Mabthera) and high dose cytarabine
- high dose BEAM chemotherapy
These treatments are followed by a transplant using your own stem cells.
You need to stay in hospital for a few weeks to have these treatments. It is hard going, but you get a lot of support from the staff during your stay in hospital.
You might have radiotherapy as a treatment on its own if you have stage 1 or 2 mantle cell lymphoma.
Or you might have radiotherapy and chemotherapy together (chemoradiotherapy) for advanced stages of the disease.
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.
Biological therapy drugs act on processes in cancer cells. They can change the way cells signal to each other, or they can stimulate your body to attack or control the growth of cancer cells.
Rituximab (Mabthera) is a type of biological therapy drug called a monoclonal antibody. You might have it with chemotherapy for mantle cell lymphoma.
Your doctor might ask if you’d like to take part in a clinical trial. Doctors and researchers do trials to:
- improve treatment
- make existing treatments better
- develop new treatments
Drugs being tested for mantle cell lymphoma include:
- alemtuzumab (MabCampath)
- lenalidomide (Revlimid)
- temsirolimus (Torisel)
- bortezomib (Velcade)