What is primary mediastinal large B-cell lymphoma?

Primary mediastinal large B-cell lymphoma (PMBCL) is a rare type of blood cancer. It is a type of lymphoma called non-Hodgkin lymphoma.

PMBCL develops when blood cells called B-cell lymphocytes become abnormal. These blood cells normally help fight infection.

The abnormal B-cells usually build up in lymph nodes behind the breastbone and between the lungs. This area is called the mediastinum. But PMBCL may also affect other lymph nodes or other areas of the body.

Booklets and resources

Symptoms of PMBCL

The symptoms of PMBCL are usually caused by the lymphoma pressing on other structures in the chest. This can cause:

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

Some of these symptoms can be caused by pressure on a large vein in the chest. This vein is called the superior vena cava (SVC). Pressure on the SVC can slow blood flow. This is called superior vena cava obstruction (SVCO).

B symptoms

PMBCL may also cause the following 3 symptoms:

  • drenching night sweats
  • unexplained high temperatures
  • unexplained weight loss.

These are called B symptoms. Along with staging and grading (below), B symptoms can help your cancer team understand:

  • how the lymphoma might develop
  • which treatments might help.

Worried about symptoms?

A change in how you feel or how your body works can be caused by conditions other than PMBCL. But always ask your GP to check any ongoing, new or unexplained symptoms. We are also here if you need someone to talk to. You can:

Related pages

Causes of PMBCL

The causes of primary mediastinal large B-cell lymphoma are not known. We have more general information about causes and risk factors for non-Hodgkin lymphoma (NHL).

Like other cancers, PMBCL is not infectious. You cannot spread it to other people.

Diagnosis of PMBCL

If you have symptoms that worry you, you should begin by going to your GP. They will examine you. If they think cancer may be causing your symptoms, they will refer you to a specialist doctor at a hospital.

The most important test for diagnosing lymphoma is a biopsy. This means testing a small piece of tissue or a sample of cells from an affected area of your body.

Find out more about having a biopsy and other tests in our general lymphoma information.

Waiting for test results

Waiting for test results can be a difficult time. It may take up to 2 weeks for the results to be ready.

You may find it helpful to talk with your partner, your family or a close friend. Your specialist nurse can also provide support.

You can talk to one of our cancer support specialists for free on 0808 808 00 00, 7 days a week, 8am to 8pm.

Booklets and resources

Staging and grading of PMBCL

Your test results give your cancer team more information about the lymphoma. This includes:

  • The stage

    Staging describes how many areas of the body are affected by lymphoma and where these areas are.

  • The grade

    Grading describes how quickly the lymphoma cells are likely to grow. PMBCL is a high grade lymphoma. High grade lymphoma grows more quickly and needs urgent treatment.

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

We have more information about staging and grading non-Hodgkin lymphoma.

Treatment for PMBCL

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

After the MDT meeting, you will usually meet with your cancer doctor and specialist nurse. They will explain the different treatment options and possible side effects. They will also talk to you about things to consider when making treatment decisions.

Your treatment options will depend on:

  • the stage of the lymphoma
  • your general health
  • your preferences.

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.

You may only need one type of treatment. Or you may need a combination of treatments.

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

The most common treatments for PMBCL are:

  • Cancer drug treatments

    PMBCL is often treated with a combination of a drug called rituximab and chemotherapy. Rituximab is a targeted and immunotherapy drug.

    Different combinations of chemotherapy may be used with rituximab, such as:

    The aim of treatment is to get rid of all signs of the lymphoma. This is called remission. If treatment is not effective or the lymphoma comes back, you may have more cancer drug treatment. These drugs are used to treat PMBCL that has come back:


  • Steroids

    Steroids are drugs that are often given with chemotherapy to treat lymphoma.


  • Radiotherapy

    Radiotherapy uses high-energy rays called radiation to treat cancer. It destroys cancer cells in the area you have it.

    Some people have radiotherapy after chemotherapy and rituximab to treat an area of lymphoma. This can also reduce the risk of lymphoma coming back in that area.


  • CAR T-cell therapy

    CAR T therapy is a treatment made from your own white blood cells. A CAR T treatment called axicabtagene ciloleucel can be used to treat PMBCL that has come back after 2 or more other treatments.


  • Stem cell transplants

    Stem cell transplants are sometimes used to treat PMBCL that has come back. A stem cell transplant is an intensive treatment, so it is not suitable for everyone.     

After PMBCL treatment

Follow-up after treatment for PMBCL

You will have regular check-ups during and after your treatment. Your cancer doctor or specialist nurse will explain what to expect.

Follow-up appointments are a good time for you to talk to your cancer doctor or specialist nurse about any concerns you have. Tell them as soon as possible if you have any problems or notice new symptoms between appointments. We have more information about follow-up care after treatment.

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

Wellbeing and recovery

Looking after yourself can help speed up your recovery after PMBCL treatment. Even small lifestyle changes may improve your wellbeing and long term health.

Even if you already have a healthy lifestyle, you may choose to make some positive changes after treatment. We have more information on leading a healthy lifestyle after treatment.

Support for people with PMBCL

People often have many different feelings during and after PMBCL treatment. If you have finished treatment, you may feel relieved it has ended but worried about what might happen in the future.

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

You can:

You may also find the HOPE programme helpful. The programme is a free 6 week self-management course designed to help you develop techniques and strategies when living with or after cancer. Topics include goal setting, fatigue management, and wellbeing.

You can sign up for the online HOPE programme. Or to find out more about face to face programmes in your area, email ServiceOpsSupport@macmillan.org.uk

Other organisations who offer information and support

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

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:

About our information

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.

  • References

    Below is a sample of the sources used in our lymphoma information. If you would like more information about the sources we use, please contact us at informationproductionteam@macmillan.org.uk

     

    Cwynarski K, Marzolini MAV, Barrington SF, et al. The management of primary mediastinal B-cell lymphoma: a British Society for Haematology Good Practice Paper. Br J Haematol. 2019;185:402-9.

     

    Eyre TA, Bishton MJ, McCulloch R, et al. Diagnosis and management of mantle cell lymphoma: A British Society for Haematology Guideline. Br J Haematol. 2024;204(1):108–26.

     

    Ferreri AJM, Illerhaus G, Doorduijn K, et al., on behalf of the EHA and ESMO Guidelines Committees. Primary central nervous system lymphomas: EHA–ESMO Clinical Practice Guideline for diagnosis, treatment and follow-up. Ann Oncol. 2024;35(6):491-507.

     

    Fox CP, Ahearne MJ, Pettengell R, et al. Guidelines for the management of mature T- and natural killer-cell lymphomas (excluding cutaneous T-cell lymphoma): a British Society for Haematology Guideline. Br J Haematol. 2022;196:507-22.

     

    Fox CP, Chaganti S, McIlroy G, et al. The management of newly diagnosed large B-cell lymphoma: A British Society for Haematology Guideline. Br J Haematol. 2024;204(4):1178–92.

     

    McNamara C, Montoto S, Eyre TA, et al. The investigation and management of follicular lymphoma. Br J Haematol. 2020;191:363-81.

     

    Pratt G, El-Sharkawi D, Kothari J, et al. Diagnosis and management of Waldenström macroglobulinaemia—A British Society for Haematology guideline. Br J Haematol. 2022;197:171–87.

     

    Walewska R, Eyre TA, Barrington S, et al. Guideline for the diagnosis and management of marginal zone lymphomas: A British Society of Haematology Guideline. Br J Haematol. 2024;204(1):86–107.

Date reviewed

Reviewed: 01 November 2024
|
Next review: 01 November 2027
Trusted Information Creator - Patient Information Forum
Trusted Information Creator - Patient Information Forum

Our cancer information meets the PIF TICK quality mark.

This means it is easy to use, up-to-date and based on the latest evidence. Learn more about how we produce our information.

The language we use


We want everyone affected by cancer to feel our information is written for them.


We want our information to be as clear as possible. To do this, we try to:

  • use plain English
  • explain medical words
  • use short sentences
  • use illustrations to explain text
  • structure the information clearly
  • make sure important points are clear.

We use gender-inclusive language and talk to our readers as ‘you’ so that everyone feels included. Where clinically necessary we use the terms ‘men’ and ‘women’ or ‘male’ and ‘female’. For example, we do so when talking about parts of the body or mentioning statistics or research about who is affected.


You can read more about how we produce our information here.