Mantle cell lymphoma: symptoms, diagnosis, treatment and outlook

Cancer Research UK says the most common symptom is one or more painless swellings in the neck, armpit or groin, which are enlarged lymph nodes. Lymphoma Action says lumps often develop in several parts of the body, many people have a swollen spleen (which can make you feel full quickly when you eat or cause discomfort behind the ribs), and many have lymphoma cells in the bone marrow at diagnosis.

The NHS lists the symptoms of non-Hodgkin lymphoma generally as a painless lump or swelling, a high temperature or feeling hot, cold or shivery, night sweats, shortness of breath, itchy skin and sudden unexplained weight loss. Many of these symptoms are common and have many causes, so having them does not definitely mean you have lymphoma.

• Painless lumps in the neck, armpit or groin, often in several parts of the body.

• Heavy night sweats, fevers that come and go, and unexplained weight loss, called B symptoms.

• Tiredness and breathlessness from anaemia, easy bruising or bleeding from low platelets, and frequent infections from low neutrophils.

• Tummy or back pain, diarrhoea or abdominal swelling if the bowel or abdomen is involved.

• Feeling full quickly or discomfort behind the ribs if the spleen is enlarged.

• Headaches, dizziness or confusion, rarely, if the central nervous system is affected.

Lymphoma is a cancer of the lymphatic system that develops when white blood cells called lymphocytes grow out of control. Mantle cell lymphoma develops from B lymphocytes, and takes its name because the abnormal cells usually develop in a part of the lymph nodes called the mantle zone.

Lymphoma Action explains that non-Hodgkin lymphomas are usually divided into fast-growing and slow-growing types, and that mantle cell lymphoma is unusual because it often has features of both. It usually grows quickly but is likely to come back after treatment, which is typical of low-grade lymphoma. Cancer Research UK says that around 5 in every 100 people with non-Hodgkin lymphoma in the UK have mantle cell lymphoma.

There are two main types. Classical mantle cell lymphoma accounts for most cases, and affects lymph nodes but often spreads to the bone marrow, spleen, bowel and liver. Leukaemic non-nodal mantle cell lymphoma is less common, with a swollen spleen and lymphoma cells in the bloodstream and bone marrow, and tends to grow slowly.

In almost all cases the cause is unknown. Lymphoma Action says most cases have a particular genetic change in the abnormal cells which makes the B cells produce too much of a protein called cyclin D1, causing them to grow out of control. Scientists do not know why this change develops, and it is not something a person causes by lifestyle choices.

Around 600 people are diagnosed each year in the UK. It is more common in men than in women, is usually diagnosed in people who are middle-aged or older, and is very rare in young people. Cancer Research UK says around 5 in every 100 people with non-Hodgkin lymphoma in the UK have mantle cell lymphoma.

Lymphoma Action says mantle cell lymphoma is typically diagnosed by a biopsy, a small procedure to remove a sample of tissue such as a swollen lymph node. In leukaemic disease it may be diagnosed through a blood test. An expert lymphoma pathologist examines the samples, looking for characteristic proteins and genetic features.

Staging tests, which might include a CT or PET/CT scan, a bone marrow biopsy, blood tests, and sometimes an MRI scan and lumbar puncture or an endoscopy depending on symptoms, find out which areas of the body are affected. Not everyone needs all of these tests.

Your doctor might also give you a prognostic score. Lymphoma Action says one of the most common in the UK is the mantle cell prognostic index, MIPI, which is based on your age, white blood cell count, the level of the protein lactate dehydrogenase in your blood and your ability to carry on with normal activities. Cancer Research UK says the score helps doctors work out whether the lymphoma is low, intermediate or high risk.

Treatment depends on the exact type of lymphoma including how quickly it is growing, the prognostic score, symptoms, stage, age and general health, and your feelings about the options. Lymphoma Action says around 1 in 10 people have a slowly growing form with few or no symptoms and might not need treatment for a long time, sometimes years, under active monitoring, sometimes called watch and wait.

For most people the lymphoma grows quickly and needs treatment straight away. For early-stage disease radiotherapy might be offered. For advanced stage disease, Lymphoma Action says the medical team is likely to suggest chemotherapy combined with an antibody therapy such as rituximab, with stronger regimens including cytarabine for people who are fit enough, sometimes followed by high-dose chemotherapy and an autologous stem cell transplant. Most people then have maintenance therapy with rituximab injections every 2 months for up to 3 years.

Lymphoma Action says mantle cell lymphoma can relapse several times, and different treatments can be used each time. Options include a targeted drug such as ibrutinib or zanubrutinib, which it describes as the most common second treatment, another targeted drug or experimental treatment in a clinical trial, a different chemotherapy regimen with rituximab, CAR T-cell therapy if you have already been treated with ibrutinib, a donor stem cell transplant in selected fit people, and supportive care.

Because treatment options change over time, it is worth asking your haematology team which are currently available to you.

Cancer Research UK says that, generally, more than 45 in 100 people (more than 45%) with mantle cell lymphoma survive their cancer for 5 years or more after diagnosis. Younger people tend to do better: almost 65 in 100 of those younger than 60, almost 55 in 100 of those aged 60 to 79, and around 20 in 100 of those aged 80 or older. These statistics are for people in one area of England diagnosed between 2010 and 2019.

Lymphoma Action points out that survival figures describe groups of people over a period of time, not your individual outlook, and are usually measured 5 years or more after treatment. It also notes that in the last few years several new treatments have become available that have improved the outlook for many people, so people diagnosed now may not have the same outlook as those in older statistics. Your medical team is best placed to advise on your own outlook, using your test results, age and fitness.

After treatment you usually have a scan to see how you have responded, then regular follow-up appointments to check recovery, give maintenance therapy, check for signs of the lymphoma coming back and look for late effects. Lymphoma Action says you are unlikely to have a scan unless you have new or worsening symptoms.

Survival statistics describe groups of people in the past, not an individual's future. Lymphoma Action points out that treatment has improved a lot in recent years, so people diagnosed now may not have the same outlook as those in older statistics, and Cancer Research UK notes that your doctor is the best person to discuss your outlook.

The NHS advises seeing a GP if you think you might have symptoms of non-Hodgkin lymphoma, because finding it early means treatment is more likely to be successful. The GP will ask about your symptoms, may feel for lumps in the neck, armpit and groin, may arrange a blood test and may make an urgent referral, which does not definitely mean you have cancer.

If you are being treated and develop a high temperature or feel very unwell, contact your haematology team or NHS 111 straight away, and call 999 for a life-threatening emergency. Your team will give you its own advice about which symptoms need same-day contact.

Lymphoma is diagnosed and treated by NHS haematology teams, with discussion at a multidisciplinary meeting and a clinical nurse specialist as the usual main contact. Lymphoma Action describes treatment including chemotherapy, targeted drugs and stem cell transplants, with clinical trials offered where suitable.

Some people ask for a private haematology opinion alongside NHS care. The UK sources used here do not describe private services or costs, so this guide does not either.