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This information is about a specific type of non-Hodgkin lymphoma (NHL)| called anaplastic large cell lymphoma.
Non-Hodgkin lymphoma is a cancer of the lymphatic system|. This 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.
The lymphatic system
View a large copy of the diagram of the lymphatic system|
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 anything that the body doesn't need. This includes bacteria, viruses, damaged cells and cancer cells.
Lymph fluid contains 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, which is where blood cells are made. The two main types of lymphocyte 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 in which either T-cells or B-cells grow in an uncontrolled way.
There are many different types of non-Hodgkin lymphomas. They're grouped (or classified) according to certain characteristics such as the type of cell involved (B-cells or T-cells). The most widely used classification system is produced by the World Health Organisation|.
Anaplastic large cell lymphoma is rare. It is more likely to affect children and young adults, and is more common in males. The lymphoma is usually made up of T-cells, although in some cases the type of cell making up the lymphoma is unclear.
Some doctors may use the terms 'ALK positive' or 'ALK negative' to describe a sub-type of anaplastic large cell lymphoma. ALK stands for anaplastic lymphoma kinase. Younger people (those under 35) are more likely to be ALK positive.
The causes of anaplastic large cell lymphoma are unknown. Like other cancers, it's not infectious and can't be passed on to other people.
The first sign of the condition is often a painless swelling in the neck, armpit or groin that is caused by enlarged lymph nodes. Sometimes lymph nodes in more than one part of the body are affected. Other symptoms may include loss of appetite| and tiredness|.
This type of lymphoma may also occur in the skin and sometimes in other organs such as the lungs, liver, bone marrow or bones.
Some people have 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 local or general anaesthetic. Biopsies may also be taken from other body tissues.
Additional tests - including blood tests, x‑rays, scans and bone marrow samples - are then used to find out more about the lymphoma and how far it has spread in the body. Doctors use this information to decide which treatment is most appropriate for you. We have more information about tests for non-Hodgkin lymphoma|.
The stage of non-Hodgkin lymphoma 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.
One group of lymph nodes is affected. 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.
Two or more groups of lymph nodes are affected. All the affected nodes are either above the diaphragm or below the diaphragm. The diaphragm is 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 specific 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; aggressive lymphomas grow more quickly.
Anaplastic large cell lymphoma is a high-grade lymphoma. This means that it is fast-growing and usually needs treatment straight away with chemotherapy.
Chemotherapy is the main treatment for anaplastic large cell lymphoma. Some people may also have stem cell treatments. Radiotherapy may be given to treat early stage disease.
This type of lymphoma usually responds well to chemotherapy and treatment may make it disappear (remission). There's a risk that the lymphoma may come back in the future. If this happens, further chemotherapy and stem cell transplants can be used to try to control it.
Chemotherapy| is the use of anti-cancer (cytotoxic) drugs to destroy cancer cells.
Anaplastic large cell lymphoma is usually treated with a combination of chemotherapy drugs. The drugs are given into a vein (intravenously).
A chemotherapy treatment called CHOP| is often used. CHOP is made up of the chemotherapy drugs vincristine|, doxorubicin|, cyclophosphamide| and the steroid| prednisolone.
More intensive combinations of drugs may be used to treat young adults and children with anaplastic lymphoma. This may mean longer stays in specialist cancer units.
Radiotherapy| uses high-energy rays to destroy cancer cells, while doing as little harm as possible to healthy cells. It may be used alone when the lymphoma cells are contained in one area of lymph nodes (stage 1). However, radiotherapy is more commonly given after chemotherapy.
Some people 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 high-dose chemotherapy with stem cell support.
Some people may have treatment using stem cells from another person (a donor). This is called a donor (allogeneic) stem cell transplant|.
Steroids| are often given with chemotherapy to help treat lymphomas. They also help you feel better and can reduce sickness and nausea|.
New treatments for anaplastic large 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. Before entering you into a trial your doctor must discuss the treatment with you and have your informed consent.
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 below for more information and support.
The Lymphoma Association| gives emotional support, advice and information on all aspects of non‑Hodgkin lymphoma. Has a national network of people with lymphoma and local groups.
Leukaemia CARE| is a national group promoting the welfare of people with leukaemia and related blood disorders, including non-Hodgkin lymphoma. Has regional support groups in many counties.
This section has been compiled using information from a number of reliable sources, including:
Guidelines for the Management of Mature T-cell and NK-cell Neoplasms (Excluding cutaneous T-cell Lymphoma) [PDF, 645kb] British Committee for Standards in Haematology 2011.
Facilities for the treatment of adults with haematological malignancies - 'Levels of Care' British Committee for Standards in Haematology 2010
Thank you to Professor Rajnish Gupta, Consultant Medical Oncologist; and all of 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.
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© Macmillan Cancer Support 2013
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