Primary mediastinal large B-cell lymphoma (PMBCL)
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- What is primary mediastinal large B-cell lymphoma (PMBCL)?
- Symptoms of primary mediastinal large B-cell lymphoma (PMBCL)
- Causes of primary mediastinal large B-cell lymphoma (PMBCL)
- Diagnosis of primary mediastinal large B-cell lymphoma (PMBCL)
- Staging and grading of primary mediastinal large B-cell lymphoma (PMBCL)
- Treatment for primary mediastinal large B-cell lymphoma (PMBCL)
- After primary mediastinal large B-cell lymphoma (PMBCL)
- About our information
- How we can help
Primary mediastinal large B-cell lymphoma (PMBCL) is a fast-growing type of non-Hodgkin lymphoma (NHL). It is also sometimes called primary thymic mediastinal lymphoma. It develops when the body makes abnormal B-cells (also called B-lymphocytes). B-cells are white blood cells that fight infection.
The abnormal B-cells (lymphoma cells) develop in a part of the lymphatic system called the thymus gland. They then build up in lymph nodes in the space behind the breast bone 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.
Symptoms may be caused by the lymphoma pressing on structures in the chest. This can cause:
- a cough
- aching in the chest
- a hoarse voice
- swelling in the neck, arm or face
- headaches that are worse when bending forward.
Some of these symptoms can be caused by lymphoma pressing on a large vein in the chest, called the superior vena cava (SVC). This vein carries all the blood from the upper half of the body to the heart. If pressure on the SVC blocks the blood from flowing along it, doctors call this superior vena cava obstruction (SVCO).
Some people also have:
- drenching night sweats
- high temperatures (fevers) with no obvious cause
- unexplained weight loss
Doctors call this group of symptoms B symptoms.
The causes of PMBCL are not known. Like other cancers, it is not infectious and cannot be passed on to other people. It mainly occurs in people aged 25 to 40, and is more common in women.
We have more information about causes and risk factors for non-Hodgkin lymphoma (NHL).
Biopsy for lymphoma
The most common test for this lymphoma is to remove part or all of an enlarged lymph node (a biopsy). This may be done under local or general anaesthetic. The biopsy is then sent to a laboratory to be checked for lymphoma cells. You may also have biopsies taken from other areas of the 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 time. It may help to talk to your family, friends or specialist nurse.
The results of your tests help your doctors find out how many areas of the 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 and high-grade lymphomas grow more quickly. PMBCL is a high-grade lymphoma and is usually diagnosed at an early stage.
We have more information about staging and grading of non-Hodgkin lymphoma.
A team of specialists will meet to discuss the best possible treatment for you. This is called a multidisciplinary team (MDT).
Your doctor or cancer specialist or nurse will explain the different treatments and their side effects. They will also talk to you about things to consider when making treatment decisions.
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 hopefully 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 or nurse before you start treatment.
Treatments for PMBCL may include the following:
The main treatment for PMBCL is chemotherapy combined with a targeted therapy called rituximab.
Commonly used treatments include:
- DA-EPOCH-R – this is made up of the chemotherapy drug etoposide, prednisolone, the chemotherapy drugs vincristine (Oncovin), cyclophosphamide and doxorubicin (hydroxydaunomycin), and rituximab. ‘DA’ stands for ‘dose-adjusted’, as the dose of drugs can be adjusted depending on how your body responds to the treatment.
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.
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.
You have regular follow-up appointments after treatment. These appointments are a good opportunity for you to talk to your doctor or nurse about any concerns you have. Your doctor will want to know how you are feeling generally, and to check you are recovering from any side effects of treatment.
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:
The organisations below also offer information and support:
Blood Cancer UK
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 email@example.com
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
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.
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