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Nodal marginal zone lymphoma is a rare type of non-Hodgkin lymphoma (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 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.
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, 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 lymphoma. They're grouped (or classified) according to certain characteristics. The most widely used classification system is produced by the World Health Organisation|.
Nodal marginal zone lymphoma (nodal MZL) is rare cancer of the B-cell. It usually affects people in their 60s and older.
Nodal means that the lymphoma starts in the lymph nodes. The marginal zone is an area of the lymph nodes. This is where the abnormal B-cells are found in nodal MZL.
The causes of nodal MZL are unknown. Like all cancers, it isn’t infectious and cannot be passed on to other people.
The first sign is often a painless swelling in the neck, armpit or groin, caused by enlarged lymph nodes. Other symptoms may include loss of appetite and tiredness (fatigue).
Some people have night sweats, unexplained 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 very small operation and may be done under local or general anaesthetic. Biopsies may also be taken from other areas of the body.
Additional tests - including blood tests, x-rays, scans and bone marrow samples - may be used to find out more about the type of lymphoma and how far it has spread in the body. Doctors use this information to decide which treatment is most appropriate for you.
You can read more about these tests in our 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, with the group being only 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 and they are all either above or below the diaphragm (the sheet of muscle under the lungs that separates the chest from the abdomen).
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. If you have night sweats, fevers or weight loss, the letter B will be added next to the stage. If you don't have these symptoms, the letter A is added.
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.
Nodal MZL is an indolent lymphoma and usually develops very slowly. In some people, it may change (transform) into an aggressive non-Hodgkin lymphoma, which means it will need more intensive treatment.
If the lymphoma is not causing symptoms, you may not need treatment immediately. Early treatment at this stage doesn’t increase a person’s life span and can cause side effects.
You will be seen regularly by your cancer specialist and treatment will be advised if you develop symptoms.
After treatment, many people have a period of time with no signs of active disease, which is known as remission. If the lymphoma comes back, it can be treated again. This can give another period of remission and the lymphoma can often be controlled in this way for many years.
Chemotherapy| is the use of anti-cancer (cytotoxic) drugs to destroy cancer cells. It is an important treatment for nodal MZL and can often get the lymphoma into remission.
Chemotherapy may be given with a drug called rituximab (Mabthera®)|, which is a monoclonal antibody that targets B-cells.
Chemotherapy can be given as tablets or into a vein (intravenously). You may be given just one type of chemotherapy drug or you may be given two or more together (combination chemotherapy).
Chemotherapy treatments for nodal MZL include:
Chlorambucil is a commonly used treatment. It comes as tablets.
CVP is a combination of the chemotherapy drugs cyclophosphamide and vincristine, and the steroid| prednisolone. It's given as a drip into a vein, usually once every three weeks. Rituximab (Mabthera®), is often given with CVP. This combination is called R-CVP|.
There are other chemotherapy drugs and combinations that can be used to treat nodal MZL. Your specialist will be able to tell you which treatment is the most appropriate for you.
Monoclonal antibodies| are drugs that recognise, target and stick to specific proteins on the surface of cancer cells, and can stimulate the body’s immune system to destroy these cells.
Rituximab is a monoclonal antibody, which targets B-cells. It may be used to treat nodal MZL. It is given as a drip into a vein (intravenous infusion).
Radiotherapy| is the use of high-energy rays to destroy cancer cells, while doing as little harm as possible to the healthy cells. It is usually given if the lymphoma cells are contained in one or two areas of lymph nodes in the same part of the body (stage 1 or 2).
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|).
Some people with lymphoma may have treatments using their own stem cells| or stem cells from a donor|. Stem cells are early blood cells that develop into all the other types of blood cell. This treatment is not suitable for everyone and is not done routinely. Doctors take into account a person's general health and age 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 a 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.
Research| into treatments for small lymphocytic leukaemia is ongoing. Cancer specialists use clinical trials to assess new treatments. Before any trial is allowed to take place, an ethics committee must have approved it and agreed that the trial is in the interest of the patients.
You may be asked to take part in a clinical trial. Your doctor will discuss the treatment with you so that you have a full understanding of the trial and what it involves. You may decide not to take part, or to withdraw from a trial at any stage. You will still receive the best standard treatment available.
You may have many different emotions| including anger, resentment, guilt, anxiety and fear. These are all normal reactions, and are part of the process many people go through in trying to come to terms with their condition.
Everyone has their own way of coping with difficult situations. Some people find it helpful to talk to family or friends, while others prefer to seek help from people outside their situation. Some people prefer to keep their feelings to themselves. There is no right or wrong way to cope, but help is there if you need it. Our cancer support specialists| can give you information about counselling in your area.
This section has been compiled using information from a number of reliable sources, including:
Thank you toProfessor 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
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© Macmillan Cancer Support 2013
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