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This information is about a type of non-Hodgkin lymphoma (NHL)| called mediastinal large B-cell lymphoma.
This section should ideally be read with our general information about NHL. We hope this information answers your questions. If you have any further questions, you can ask your doctor or nurse at the hospital where you are having treatment.
Non-Hodgkin lymphoma (NHL) is a cancer of the lymphatic system|. The lymphatic system is part of the body’s immune system and helps us fight infection. It's made up of organs such as the bone marrow, thymus, spleen, and the lymph nodes (or lymph glands). Lymph nodes are connected by a network of tiny lymphatic vessels that contain lymph fluid. There is also lymphatic tissue in other organs, such as the skin, lungs and stomach.
There are lymph nodes all over the body. As lymph fluid flows through the lymph nodes, the nodes collect and filter out anything harmful or that the body doesn't need. This includes bacteria, viruses, damaged cells and cancer cells.
The lymphatic system
View a large copy of the diagram of the lymphatic system|
Lymph fluid contains cells called lymphocytes. These are a type of white blood cell that help the body fight infection and disease.
Lymphocytes start to grow in the bone marrow, where blood cells are made. The two main types of lymphocytes are B-cells and T-cells. B-cells mature in the bone marrow, while T-cells mature in the thymus gland behind the breast bone. When they're mature, both B-cells and T-cells help fight infections.
Lymphoma is a disease where either T-cells or B-cells grow in an uncontrolled way.
There are many different types of NHL. The types are grouped (or classified) according to certain characteristics. The most widely used classification system is produced by the World Health Organisation|.
Mediastinal large B-cell lymphoma is a rare type of diffuse large B-cell lymphoma|. It starts as a growth (mass) in the mediastinum.
The mediastinum is an area inside the chest, between the lungs and behind the breast bone. It contains the heart, some large blood vessels, the gullet, the windpipe, the thymus and lymph nodes.
MLBCL usually affects younger people aged 25-40, and is more common in women than in men.
The causes of MLBCL are unknown.
It is not infectious and cannot be passed on to other people.
Symptoms may be caused by the lymphoma pressing on nearby structures in the chest. This can cause a cough, aching in the chest, or breathlessness|. Sometimes it may cause hoarseness. Some people develop swelling in their neck, arm and face, due to the lymph nodes pressing on the veins in the chest. This is known as SVCO (superior vena cava obstruction)|.
Lymph nodes in the neck, armpit or groin may also be affected and cause a painless swelling. Other symptoms include loss of appetite and tiredness.
Some people have more general symptoms, such as night sweats, high temperatures (fevers) and weight loss. These are known as B symptoms.
A diagnosis is made by removing an enlarged lymph node (a biopsy) and examining the cells under a microscope. It is a small operation and may be done under a local or general anaesthetic.
Sometimes a biopsy is taken during a CT scan, which helps the doctor make sure the biopsy is taken from the affected area.
If the lymph nodes in the mediastinum are the only ones affected, the biopsy is likely to be taken during a procedure known as a mediastinoscopy. The doctor makes a small cut in the chest and inserts a thin, flexible tube with a camera attached so that tissue samples of the lymph nodes can be taken.
Additional tests include:
The results of these tests are used to find out more about the lymphoma and how far it has spread in the body. This information is used to help decide which treatment is most appropriate. We have more information about tests for non-Hodgkin lymphoma|.
The stage of NHL describes how many groups of lymph nodes are affected, where they are in the body, and whether other organs such as the bone marrow or liver are involved. A group of lymph nodes refers to lymph nodes in one area of the body, such as in the armpit, on one side of the neck, or in the groin.
There are four stages:
One group of lymph nodes is affected.
Two or more groups of lymph nodes are affected, and all the affected lymph nodes are either above or below the diaphragm (a sheet of muscle under the lungs).
The lymphoma is in lymph nodes both above and below the diaphragm.
The lymphoma has spread beyond the lymph nodes to other organs, such as the bones, liver or lungs.
As well as giving each stage a number, doctors also use either the letter A or B to show whether or not you have any of the following symptoms: weight loss, fevers or night sweats.
If you don't have any of these symptoms, the letter A will be added next to the stage. If you do have these symptoms, the letter B is added next to the stage.
Sometimes the lymphoma can start in areas outside the lymph nodes. This is called extranodal lymphoma, and the stage will include the letter E (for extranodal).
For practical purposes, non-Hodgkin lymphomas are divided into two groups: indolent (sometimes called low-grade) and aggressive (sometimes called high-grade). Indolent lymphomas are usually slow-growing, and aggressive lymphomas grow more quickly.
Mediastinal large B-cell lymphoma is an aggressive lymphoma. This means that it is fast-growing and usually needs treatment straight away with chemotherapy.
Chemotherapy is the main treatment for mediastinal large B-cell lymphoma. Some people may also have stem cell treatments.
Other treatments are also used, such as monoclonal antibodies, steroids and radiotherapy. You may have a combination of different treatments.
Mediastinal large B-cell lymphoma usually responds well to chemotherapy, but in some people the lymphoma comes back and further treatment is needed.
Chemotherapy| is the use of anti-cancer (cytotoxic) drugs to destroy cancer cells. You will usually have a combination of different chemotherapy drugs| given into the vein (intravenously), either as injections or through a drip (infusion). Your specialist will explain which combination of drugs is most appropriate for you.
A commonly used chemotherapy combination is the R-CHOP| regimen. This includes the chemotherapy drugs vincristine|, cyclophosphamide|, doxorubicin|, the steroid| prednisolone, and a monoclonal antibody called rituximab| (Mabthera®).
Another chemotherapy combination that may sometimes be used is DA-EPOCH-R. This includes the chemotherapy drugs etoposide|, vincristine, cyclophosphamide and doxorubicin, the steroid prednisolone, and the monoclonal antibody rituximab.
Monoclonal antibodies| are drugs that recognise, target and stick to particular proteins on the surface of cancer cells, and can stimulate the body’s immune system to destroy these cells.
Rituximab| is the main monoclonal antibody drug used to treat mediastinal large B-cell lymphoma. It's given as a drip (infusion). Rituximab is usually given with chemotherapy as part of the R-CHOP regimen.
Some people with lymphoma may have treatments using their own stem cells| or stem cells from a donor|. Stem cells are a special type of blood cell that can make all other types of blood cells.
This treatment is not suitable for everyone and is not done routinely. Doctors take into account a person's general health and fitness before recommending them.
Some people have some of their own stem cells collected and stored. This allows them to have higher doses of chemotherapy to destroy the lymphoma cells.
After the chemotherapy their stem cells are returned by a drip (like a blood transfusion) to help their blood cells recover from the effects of chemotherapy. This is called an autologous transplant.
Some people may have treatment using stem cells from another person (a donor). This is called an allogeneic transplant.
Radiotherapy| is the use of high-energy rays to destroy cancer cells while doing as little harm as possible to the healthy cells. After the chemotherapy, radiotherapy is sometimes given to the mediastinal area.
Steroids| are drugs that are often given with chemotherapy to help treat lymphomas. They also help you feel better and can reduce feelings of sickness (nausea|).
Sometimes there is a risk of developing scar tissue in the affected lymph nodes after treatment for this type of lymphoma. The scar tissue can make it difficult to assess whether or not there are still lymphoma cells in the lymph glands.
Sometimes a scan known as a PET scan| may be used after treatment is finished to check whether there's any active lymphoma still present.
New treatments for mediastinal large B-cell lymphoma are being researched all the time. Your doctor may invite you to take part in a clinical trial| to compare a new treatment against the best available standard treatment. They must discuss the treatment with you and have your informed consent before entering you into a trial.
Before any trial is allowed to take place, it must be approved by a research ethics committee, which protects the interests of those taking part.
You may decide not to take part, or to withdraw from the trial at any stage. You will then receive the best standard treatment available.
Everyone has their own way of dealing with their 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. You can also contact our cancer support specialists| or the organisations listed here for more information and support.
This section has been compiled using information from a number of reliable sources, including:
Thank you to Professor Rajnish Gupta, Consultant Medical Oncologist, and the people affected by cancer who reviewed this edition. Reviewing information is just one of the ways you could help when you join our Cancer Voices network|.
Content last reviewed: 1 January 2013
Next planned review: 2015
For answers, support or just a chat, call the Macmillan Support Line free (Monday to Friday, 9am-8pm)
If you have any questions about cancer, need support or just want someone to talk to, ask Macmillan.
Professor Tony Goldstone explains how lymphomas develop and possible treatments.
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© Macmillan Cancer Support 2013
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