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.

How common is mantle cell lymphoma?

Each year around 600 people are diagnosed with mantle cell lymphoma in the UK. Out of all people with NHL in the UK, around 5 in every 100 people (around 5%) have mantle cell lymphoma.

Symptoms of mantle cell lymphoma

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

Painless swellings

The most common symptom 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
• high 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. It is important to tell your doctor about any symptoms like this.

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

Getting diagnosed

The main test to diagnose mantle cell lymphoma is a biopsy .  A doctor removes a sample from the affected area. This often means removing part or all of a swollen lymph node. They send it to the laboratory for a specialist to look at it under a microscope. They might also take biopsies from other areas of your body.

You usually also have some blood tests.

If your doctor diagnoses mantle cell lymphoma, you usually have further tests. These might include:

• a PET-CT scan   
• a bone marrow test   to check if you have lymphoma cells in your bone marrow

if your doctor thinks lymphoma might be affecting your brain or spinal cord you might have:

• an MRI scan to look at your head 
• a lumbar puncture  to check for lymphoma cells in the fluid around your brain and spinal cord

You might have an endoscopy if your doctor thinks your lymphoma might be affecting your digestive system.  This is when the doctor passes a thin tube (endoscope) through your mouth or your back passage. They can look at your digestive system and take biopsies of any abnormal looking tissue.

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. 

Your risk of mantle cell lymphoma coming back (outlook)

Your doctor might use test results to give you a prognostic score. The score is based on several factors. This helps doctors to talk to you about your prognosis. And to predict how you might respond to treatment. They call it the mantle cell prognostic index, or MIPI. They look at the following factors:

• your age
• your white blood cell count
• how lymphoma affects your well being, also known as performance status
• the level of an enzyme called lactose dehydrogenase (LDH) in your blood

These factors score points to give an overall score. This score helps your doctors work out whether your lymphoma is low, intermediate (medium), or high risk. 

Survival

The best person to talk to you about your outlook or prognosis is your doctor. Not everyone wants to know. People cope differently with their lymphoma and want different information.

Survival depends on many factors. So no one can tell you exactly how long you will live. Your doctor might be able to give you some guide, based on their knowledge and experience.

Treatment for mantle cell lymphoma

Mantle cell lymphoma is most often at an advanced stage at diagnosis. It sometimes progresses slowly. But it can also behave more aggressively and progress rapidly.

Treatment can sometimes get rid of the lymphoma completely. But unfortunately it can come back fairly soon afterwards.

Your treatment depends on:

• where your lymphoma is (the stage)
• your symptoms
• your general health and fitness
• genetic tests on your lymphoma cells

The main treatments

The main treatment options for mantle cell lymphoma are:

• chemotherapy combined with steroids and a targeted cancer drug - doctors call this chemoimmunotherapy
• stem cell transplant
• a targeted cancer drug called rituximab on its own, to stop your lymphoma from coming back

After treatment, NHL sometimes doesn't go away or comes back. Your specialist might suggest other types of treatment in this situation including:

• chemoimmunotherapy
• targeted cancer drugs  
• CAR-T cell therapy

For early stage mantle cell lymphoma, you might have radiotherapy on its own as your main treatment. This isn't common.

Treatment for early stage lymphoma (stage 1 and 2)

You might not have treatment straight away if your lymphoma is slow growing and your symptoms aren't causing problems. You have regular check ups instead. This is called active monitoring, or watch and wait.

How often you have check ups depends on your situation.  At your appointment:

• the doctor exams you
• you have blood tests

Your doctor might suggest you start treatment when you have symptoms or your lymphoma shows signs of progressing.

In very rare situations, you might have radiotherapy to treat early stage mantle cell lymphoma. Radiotherapy uses high energy waves similar to x-rays to kill cancer cells. You have radiotherapy to the affected lymph nodes. 

Treatment for advanced lymphoma - if you're fit enough for intensive treatment

Advanced stage lymphoma usually means stage 3 and stage 4. 

Your first treatment is usually a combination of chemotherapy drugs, targeted cancer drugs and steroids. Doctors call this chemoimmunotherapy.

You might then have a stem cell transplant, depending on your situation. This is followed by rituximab maintenance treatment. If you don't have a stem cell transplant you might have rituximab maintenance instead. 

Chemoimmunotherapy

If you are fit enough, common chemoimmunotherapy combinations are R-CHOP alternating with either R-DHAP or rituximab-cytarabine.

• R-CHOP is rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone 
• R-DHAP is rituximab, cytarabine, cisplatin and dexamethasone

This is often called the Nordic protocol.

You have the chemoimmunotherapy drugs on certain days. This is usually over 3 weeks. Each 3 week period is called a cycle of treatment. How many cycles you have depends on your situation. It's common to aim for 6 cycles.

Stem cell transplant

You might have a stem cell treatment if you are fit enough. And if your lymphoma has responded to chemoimmunotherapy. 

Your doctor might not offer you a transplant if your lymphoma has high risk features. There have been recent advances in understanding mantle cell lymphoma. And there is evidence that a transplant might not work as well for some people. Your healthcare team will discuss this with you.

A stem cell transplant allows you to have very high doses of chemotherapy. You then have a transplant using your own stem cells. This is called an autologous stem cell transplant.

There are different combinations of high dose chemotherapy. These include:

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

Maintenance treatment

After your stem cell transplant, you might have further treatment. This is to try to delay the lymphoma coming back. It is called maintenance treatment. You might have rituximab every 2 months for 2 to 3 years.

Treatment for advanced lymphoma - if you're not fit enough for intensive treatment

If you are not fit enough to have a transplant, you might have chemoimmunotherapy on its own. You might have:

• R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and dexamethasone)
• bendamustine and rituximab
• R-BAC (rituximab, bendamustine and cytarabine)
• VR-CAP (bortezomib, rituximab, cyclophosphamide, doxorubicin, and prednisone) 

You might have maintenance therapy after this to control your lymphoma for as long as possible. This is usually an injection of rituximab every 2 months for 2 to 3 years.

You might not be fit enough to have these  treatments. If this is the case, you might have one of the following treatments:

• chlorambucil chemotherapy tablets with rituximab
• R-CVP (rituximab, cyclophosphamide, vincristine, and prednisone)

Treatment when lymphoma doesn't go away or comes back

Lymphoma that does not go away with treatment is called refractory lymphoma. Lymphoma that comes back after treatment is called relapsed disease. Your treatment depends on what treatment you have already had, and how long your lymphoma was under control. It also depends on your general health. ​​

You might have:

• chemotherapy with rituximab
• a targeted drug called ibrutinib
• CAR T-cell therapy
• an allogenic stem cell transplant - this uses stem cells from a donor 

For CAR T-cell therapy a specialist collects and makes a small change to your T cells. After a few weeks, you have a drip containing these cells back into your bloodstream. The CAR T-cells then recognise and attack the cancer cells. 

You might have it if your lymphoma has continued to grow or relapsed following treatment with ibrutinib. You need to be well enough for this treatment. It is a very intensive treatment. Some people are too unwell to receive it.

Treating and preventing problems caused by lymphoma

Lymphoma and its treatment can cause problems. Supportive treatments can help to either prevent or control these problems.  

Supportive treatments you might need include:

• medicines to prevent bacterial or viral infections
• injections to boost your immune system (GCSF)

Follow up

You have regular follow up appointments after finishing treatment. These are appointments to check how you are. And to check there are no signs of the lymphoma coming back.  

Your doctor will examine you and ask about any symptoms or side effects. You usually have blood tests. 

You can ask any questions at these appointments. Contact your health care team between appointments if you have any symptoms or concerns. You don't need to wait for a booked appointment.

Research

Researchers around the world are looking at the treatments for NHL. Your doctor might suggest you take part in a clinical trial. 

Coping with mantle cell lymphoma

Mantle cell lymphoma and its treatment can cause physical changes in your body. Some of the treatments can be very intense. You might be in and out of hospital for a few months. These changes can be hard to cope with and can affect the way you feel about yourself.

You might also have to cope with feeling very tired and lethargic a lot of the time, especially for a while after treatment.

Ask your medical team about possible support and help.

It can help to talk to friends and family. Or join a support group to meet people in a similar situation.

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