What is primary mediastinal large B-cell lymphoma?

Primary mediastinal large B-cell lymphoma (PMBCL) is a type of non-Hodgkin lymphoma (NHL). It is sometimes called primary thymic mediastinal lymphoma.

PMBCL develops when B-cells become abnormal (cancerous). B-cells are white blood cells that normally help fight infection. They are sometimes called B-lymphocytes.

The abnormal B-cells (lymphoma cells) usually develop in a part of the lymphatic system called the thymus gland. They then build up in lymph nodes behind the breastbone and between the lungs. This area is called the mediastinum. It contains:

the gullet
the windpipe
the thymus
the heart
some large blood vessels
some lymph nodes.

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For healthcare professionals

If you are a healthcare professional, use our guide to find the right information and support for your patients affected by lymphoma. This explains the support available from Macmillan and from other trusted organisations.

Symptoms of primary mediastinal large B-cell lymphoma

Symptoms of primary mediastinal large B-cell lymphoma (PMBCL) may be caused by the lymphoma pressing on structures in the chest. This can cause:

a cough
aching in the chest
breathlessness
a hoarse voice
swelling in the neck, arm or face
dizziness
headaches that are worse when bending forward.

Some of these symptoms can be caused by lymphoma pressing on a large vein in the chest. This vein is called the superior vena cava (SVC). It carries all the blood from the upper half of the body to the heart. Pressure on the SVC can block blood from flowing along it. Doctors call this superior vena cava obstruction (SVCO).

B symptoms

Some people also have symptoms that doctors call B symptoms. These can include:

drenching night sweats which require a change of nightwear and bed covers
high temperatures (fevers) with no obvious cause
unexplained weight loss.

Knowing if you have any B symptoms will help your doctor to stage the lymphoma and plan your treatment.

Causes of primary mediastinal large B-cell lymphoma

The causes of primary mediastinal large B-cell lymphoma (PMBCL) are not known.

Like other cancers, PMBCL is not infectious. It cannot be passed on to other people.

We have more information about causes and risk factors for non-Hodgkin lymphoma (NHL).

Diagnosis of primary mediastinal large B-cell lymphoma

Biopsy for lymphoma

The most common test for diagnosing lymphoma is a biopsy. A doctor will take a sample of tissue from the affected area. The most common place to take a biopsy from is an enlarged lymph node. This is called a lymph node biopsy). You may have all or a part of the lymph node removed. The tissue will be sent to a laboratory for testing. You may also have biopsies taken from other areas of your body.

You can read more about further tests you may have in our information about non-Hodgkin lymphoma.

Waiting for test results can be a difficult. You may find it helpful to talk to your family, friends or specialist nurse.

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Booklets and resources

The stages and grades of primary mediastinal large B-cell lymphoma

Your test results will help your doctors find out how many areas of your body are affected by lymphoma and where these areas are. This is called staging. Doctors may use the term bulky disease to describe tumours in the chest that are 10cm (4 inches) across or bigger.

Lymphomas are also grouped as either low-grade or high-grade. Low-grade lymphomas are usually slow growing. High-grade lymphomas usually grow more quickly. Primary mediastinal large B-cell lymphoma (PMBCL) is a high-grade lymphoma.

Knowing the stage and grade of the lymphoma helps your doctor plan the right treatment for you.

We have more information about the stages and grades of non-Hodgkin lymphoma.

Treatment for primary mediastinal large B-cell lymphoma

A team of specialists will meet to discuss the best possible treatment for you. They are called a multidisciplinary team (MDT).

Your doctor, or cancer specialist or nurse will explain the different treatments and their side effects to you. They will also talk to you about things to consider when making treatment decisions.

Primary mediastinal large B-cell lymphoma (PMBCL) is fast-growing and needs to be treated quickly. The aim of treatment is to get rid of all signs of the lymphoma and to cure you.

Some treatments for PMBCL can affect whether you can get pregnant or make someone pregnant. If you are worried about this, it is important to talk with your doctor, cancer specialist or nurse before you start treatment.

Treatment for PMBCL depends on the stage of the lymphoma and whether you have symptoms that are causing problems.

The most common treatments for PMBCL are:

Immunotherapy and chemotherapy
PMBCL is often treated with a combination of the immunotherapy drug rituximab and chemotherapy. This is called chemoimmunotherapy. Commonly used combinations of chemoimmunotherapy for PMBCL include:

R-CHOP

DA-EPOCH-R – etoposide, prednisolone, vincristine (Oncovin), cyclophosphamide and doxorubicin (hydroxydaunomycin), and rituximab. ‘DA’ stands for ‘dose-adjusted’. This means that the dose of drugs can be adjusted depending on how your body responds to the treatment.
Steroids

Steroids are drugs that are often given with chemotherapy to treat lymphomas. They help make chemotherapy more effective.
Radiotherapy

Radiotherapy uses high-energy rays to destroy cancer cells, while doing as little harm as possible to normal cells. Some people have radiotherapy after chemotherapy and rituximab to treat an area of lymphoma. It can also reduce the risk of lymphoma coming back in that area.
Stem cell transplants

Stem cell transplants are sometimes used to treat lymphoma that has come back after treatment. Stem cell transplants are intensive treatments, so they are not suitable for everyone. You may have a transplant using your own stem cells autologous stem cell transplant or cells from a donor allogeneic stem cell transplant.
CAR-T cell therapy

CAR-T cell therapy is sometimes used to treat PMBCL that has come back after treatment. CAR-T therapy (chimeric antigen receptor therapy) involves collecting your own immune cells and reprogramming them. The cells are then used as a treatment to target the lymphoma cells. CAR-T therapy can be used when the lymphoma is advanced and when other treatments have failed. CAR-T therapy is complicated and can have serious side effects. It is only available in a few specialist hospitals.

You may have some treatments as part of a clinical trial.

PMBCL usually responds well to treatment. But if it does not respond or if the lymphoma comes back, you will need more treatment.

We have more information about treating lymphoma that has come back.

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After treatment for primary mediastinal large B-cell lymphoma

People often have many different feelings when they finish lymphoma treatment. You may feel relieved that treatment has finished, but worried about what will happen in the future.

You will have appointments with your lymphoma doctor or nurse less often than before. But at the same time, you may have new challenges to cope with and things to think about.

We have information below about some of the things people ask about after lymphoma treatment. But you may have other questions or need information about something else. If there is something you want to talk about at any point after treatment, you can:

call the Macmillan Support Line free on 0808 808 00 00
chat to our specialists online
visit our Online Community to talk to people who have been affected by lymphoma, share your experience, and ask an expert your questions.

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After treatment

Lymphoma follow-up

You will have regular follow-up appointments after your treatment. You can talk to your doctor or nurse about any concerns you have at these appointments. Your doctor will want to know how you are feeling, and to check you are recovering from any side effects of treatment.

Related pages

Follow-up after lymphoma treatment

Side effects of lymphoma treatment

You may have some ongoing side effects as you recover from lymphoma treatment. You can use our impacts of cancer A-Z to search for information about managing different symptoms and side effects. Or find out more about side effects of treatment for Hodgkin lymphoma or treatment for non-Hodgkin lymphoma.

Tiredness and fatigue

Tiredness (fatigue) often affects people with cancer. It can be caused by lymphoma or be a side effect of lymphoma treatment. RESTORE is an online resource that aims to help people living with cancer related fatigue.
Sexual well-being

Lymphoma and its treatment can sometimes affect your sex life. There are ways to improve your sexual well-being and to manage any problems.
Fertility
Treatment for lymphoma may affect your fertility. If you are worried about your fertility it is important to talk with your doctor before you start treatment. We have more information about:

fertility after treatment for women

fertility after treatment for men.

Sometimes side effects may continue or develop months or years after treatment. These are called late effects. We have more information about long-term and late effects of treatment for lymphoma.

Well-being and recovery

It can take time to recover after lymphoma treatment. Some days you may feel better than others.

It is important to know where to get support or information if you need it. People often need support even some time after lymphoma treatment. But sometimes it is difficult to know who to ask for help. To find support:

ask your GP or someone from your cancer team for advice about support in your area
search cancercaremap.org to find cancer support services near you
call us free on 0808 808 0000 or talk to us online - our cancer information and support specialists can offer guidance and help you find what you need.

Our course Help to Overcome Problems Effectively (HOPE) helps people during and after cancer treatment. It is a free, interactive, group based, self management support course. It runs for 6 weeks, with each weekly session lasting 2.5 hours. To find out more about HOPE courses in your area, email ServiceOpsSupport@macmillan.org.uk

A healthy lifestyle can help speed up your recovery. Even small lifestyle changes may improve your well-being and long-term health.

Related pages

Being physically active after treatment

Booklets and resources

Support for people with primary mediastinal large B-cell lymphoma

We understand that being diagnosed with cancer can affect you both physically and emotionally. We have information and advice to help you.

Talk to someone about primary mediastinal large B-cell lymphoma

Everyone has their own way of dealing with illness and the different emotions they experience. You may find it helpful to talk things over with family and friends or your doctor or nurse.

Macmillan is also here to support you. If you would like to talk, you can:

Call the Macmillan Support Line on 0808 808 00 00.
Chat to our specialists online.
Visit our non-Hodgkin lymphoma forum to talk with people who have been affected by non-Hodgkin lymphoma (NHL), share your experience, and ask an expert your questions.

More information and advice

We know cancer can affect you physically, emotionally and financially. We have information and advice about different ways cancer might impact you, such as help with:

Other organisations who offer information and support

The organisations below also offer information and support:

Blood Cancer UK

Blood Cancer UK offers support and information to people affected by blood cancers, including lymphoma.
Lymphoma Action

Lymphoma Action provides high quality information and support for people affected by lymphoma. It provides helpline services and a range of peer support including online support meetings, educational events and webinars. Its website includes TrialsLink, a database of lymphoma clinical trials.

Supporting someone with lymphoma

When someone you know is diagnosed with lymphoma, it can be difficult to know how to support them. You may want information to help you understand what they are going through. Or you may be worried about what to say.

We have information to help with the practical, emotional and financial impacts of supporting someone. You can also talk to us by:

calling the Macmillan Support Line
using chat online
visiting our family and friends forum to talk with people who are supporting someone with cancer, share your experience, and ask an expert your questions.

Booklets and resources

Access our lymphoma information in other formats

We are working to make our website as accessible as possible. We want everyone to be able to use it to find the information they need. We have tips about using settings on your computer or device to help you use our website in our accessibility statement.

We also provide information in a range of languages and formats. If you cannot find the information you are looking for in the format or language you need, email us at cancerinformationteam@macmillan.org.uk

Order our non-Hodgkin lymphoma booklet

Download our lymphoma booklet and ebooks

Our Understanding non-Hodgkin lymphoma booklet is available as a pdf to download or view online and in ePub or Mobi formats to download.

Booklets and resources

Listen to our lymphoma audiobook

You can listen to our Understanding non-Hodgkin lymphoma audiobook. You can also search our full range of audiobooks.

Booklets and resources

Find non-Hodgkin lymphoma information in your language

We have a range of translated cancer information. This includes information about different cancer types, being diagnosed, cancer treatment, and side effects. We have some lymphoma information in the following languages. You can also search our most up to date list of web pages we have translated on request.

Bulgarian - Mantle cell lymphoma / Мантелноклетъчен лимфом [PDF]
German - Follicular lymphoma / Follikuläres Lymphom (FL) [PDF]
Polish - Diffuse large B-cell lymphoma (DLBCL) / Chłoniak rozlany z dużych komórek B [PDF]
Polish - Lymphoma / Chłoniak [PDF]
Slovak - Anaplastic large cell lymphoma (ALCL) / Anaplastický veľkobunkový lymfóm [PDF]
Slovak - Follicular lymphoma / Folikulový lymfóm [PDF]
Tamil - Follicular lymphoma [PDF]

If you would like any of our lymphoma information translated into your language, please email cancerinformationteam@macmillan.org.uk

Watch British sign language videos

You can watch our BSL videos about cancer on YouTube.

Find our easy read booklets

Our easy read booklets use simple words and pictures to tell you about cancer. They can be useful if you want information that is easier to understand.

Looking for large print, Braille or another format?

If you would like our information in a different format such as large print or Braille, email us at cancerinformationteam@macmillan.org.uk or call us free on 0808 808 00 00.

About our information

References

Below is a sample of the sources used in our primary mediastinal large B-cell lymphoma (PMBCL) information. If you would like more information about the sources we use, please contact us at cancerinformationteam@macmillan.org.uk

Vitolo U, Seymour JF, et al. Extranodal diffuse large B-cell lymphoma and primary mediastinal B-cell lymphoma: ESMO Clinical Practice Guidelines. Annals of Oncology. 2016. 27 (Supplement 5), v91-v102. Available from: www.annalsofoncology.org/article/S0923-7534(19)31640-0/pdf

Reviewers

This information has been written, revised and edited by Macmillan Cancer Support’s Cancer Information Development team. It has been reviewed by expert medical and health professionals and people living with cancer. It has been approved by Senior Medical Editor, Professor Rajnish Gupta, Macmillan Consultant Medical Oncologist.

Our cancer information has been awarded the PIF TICK. Created by the Patient Information Forum, this quality mark shows we meet PIF’s 10 criteria for trustworthy health information.