What is mantle cell lymphoma?

Lymphoma is a type of blood cancer. If you have any type of blood cancer, it means some of your blood cells are not working properly.

There are different types of blood cell in the body – red blood cells, white blood cells and platelets. They all do different, important jobs.

In lymphoma, it is your lymphocytes that are affected. Lymphocytes are a type of white blood cell, and they help your body fight infections.

There are two main types of lymphoma – Hodgkin lymphoma and non-Hodgkin lymphoma. Mantle cell lymphoma is a type of non-Hodgkin lymphoma.

There are two types of lymphocytes – B-cell lymphocytes and T-cell lymphocytes. Mantle cell lymphoma affects B-cell lymphocytes.

Mantle cell lymphoma develops in the lymph nodes (glands), in a specific part called the mantle zone. When you have mantle cell lymphoma, abnormal lymphocytes begin to build up in the mantle zone, forming swellings or lumps.

These abnormal lymphocytes can also get into the blood and lymphatic system – the system of tiny vessels that carry a fluid called lymph around your body. This means the lymphoma often affects more than one lymph node and can cause swellings in other organs such as the bowel (gut) or spleen (part of your lymphatic system).

Read more about the lymphatic system.

Doctors debate whether mantle cell lymphoma is a high grade (fast growing) or low grade (slow growing) disease. Under the microscope, mantle cell lymphoma looks like a low grade condition. But it can behave like a high grade condition, developing very quickly.

There are two main types of mantle cell lymphoma. These develop at different speeds and in different places:

In classical mantle cell lymphoma, lumps form in the lymph nodes, and often in other organs such as the blood, bowel, stomach or spleen. This is the most common type of mantle cell lymphoma and it typically develops quickly, although there are some cases that develop slowly.

Some very fast growing types of classical mantle cell lymphoma include pleomorphic mantle cell lymphoma and blastoid mantle cell lymphoma.

In this type of mantle cell lymphoma, the lymph nodes are not usually swollen. Instead, abnormal lymphocytes mainly gather in the bloodstream, bone marrow (the spongy tissue inside your bones where blood cells are made) and the spleen.

Leukaemic non-nodal mantle cell lymphoma usually develops slowly. Your doctor may call it “indolent”. It is much less common than classical mantle cell lymphoma.

Because it is very rare, there is limited information on the prognosis for mantle cell lymphoma – what will happen in the future.

So far, research has shown that treatment can usually get rid of mantle cell lymphoma but that it is likely to come back later. It’s common for people with mantle cell lymphoma to go through cycles of remission, where no cancer is present, and relapse, where the cancer returns and more treatment is needed.

As research progresses, treatments are improving and many more people are able to enjoy a good quality of life through longer periods of remission.

Some people find it helpful to know as much as possible about their personal prognosis, others prefer not to think too much about the future. If you want to know more about your own situation, your hospital team are the best people to ask as they know most about your condition.

You can also read our general information about blood cancer prognosis, including how to understand statistics you may see online.

Find out more about our lymphoma research, bringing hope for better, kinder treatments in the future.

Around 500 people in the UK are diagnosed with mantle cell lymphoma each year. It typically affects older people, with an average age at diagnosis around 70 years old. It is more much common in men than women.

We don’t yet know exactly what causes mantle cell lymphoma. We know it is not caused by lifestyle choices, so it is not because of anything you have done.

Researchers have found some links between genetic changes and mantle cell lymphoma. In every cell in your body, there are genes. Genes instruct the cell to carry out certain tasks to keep us alive and healthy. Changes to genes (known as mutations) make cells act abnormally.

There is a connection between mantle cell lymphoma and a particular genetic mutation where two chromosomes (long structures of DNA that carry genes) swap pieces of information. This is known as a translocation.

In mantle cell lymphoma, parts of chromosome 11 swap with parts of chromosome 14. This mutation is referred to as t(11;14). It is linked to the overproduction of a protein called cyclin D1. Too much cyclin D1 can cause lymphocytes to grow too fast and in an abnormal way, usually leading to cancer.

Around 9 in 10 people with mantle cell lymphoma (90%) have the t(11;14) genetic mutation. People who don’t have this mutation usually have another genetic change that leads to too much cyclin D1 being produced.

The type of mantle cell lymphoma you have is also linked to genetic mutations. Classical mantle cell lymphoma has been linked with a mutation of the SOX11 gene, and leukaemic non-nodal mantle cell lymphoma has been linked with a mutation of the IGHV gene.

All of these possible genetic mutations happen throughout your lifetime – they are not inherited, meaning you cannot get mantle cell lymphoma from your parents or give it to your children.