Freephone helpline 0808 808 5555 information@lymphomas.org.uk www.lymphomas.org.uk (nodal MZL) is a rare, slow-growing cancer of the lymphatic system, which is part of the body's immune system. It is a type of non-hodgkin lymphoma. It usually affects people in their 50s and 60s but it can occur in younger and older people too. In this information sheet we aim to answer the questions you might have about this lymphoma: What is nodal MZL? (see below) What causes nodal MZL? (page 3) What are the symptoms of nodal MZL? (page 3) What tests will I need? (page 3) What are the stages of nodal MZL? (page 4) How is nodal MZL treated? (page 5) What happens if nodal MZL comes back? (page 6) We have separate information on other types of marginal zone lymphomas. If you would like information on splenic MZL or MALT lymphoma, please ring our helpline on 0808 808 5555 and they can send you this. Alternatively, you can download the information from our website (www.lymphomas.org.uk). What is nodal MZL? What is lymphoma? A lymphoma is a cancer of cells called lymphocytes, which are white blood cells that normally help the body to fight infections. There are two kinds of lymphocyte, B lymphocytes (usually just known as 'B cells') and T lymphocytes (T cells). B cells are made in the bone marrow, which is a spongy tissue found in the middle of some of our bigger bones. T cells start their life in the bone marrow but they mature in the thymus, a gland in the chest which lies behind the sternum or breastbone. Lymphocytes collect in lymph nodes (glands). Lymph nodes are found in groups, particularly under the arms, in the neck and in the groin. We also have groups of lymph nodes internally, around our organs. Lymphocytes can also be found in other parts of the body, such as in the spleen and the thymus. These places are all part of our lymphatic system (see Figure 1 on page 2). If lymphocytes start to divide and multiply uncontrollably or if they don t die off after their normal lifespan, they can build up and form a lymphoma. 1/8
Figure 1: The lymphatic system Neck (cervical) lymph nodes Lymph vessels Armpit (axillary) lymph nodes Groin (inguinal) lymph nodes Thymus Diaphragm (muscle that separates the chest from the abdomen) Spleen Liver What types of lymphoma are there? Lymphomas can develop from either type of lymphocyte. There are therefore B-cell lymphomas and T-cell lymphomas. If a lymphoma develops and grows quickly it is called a high-grade or aggressive lymphoma. If it is slow-growing it is a low-grade or indolent lymphoma. There are over 60 types of lymphoma, but there are two main kinds Hodgkin lymphoma and non-hodgkin lymphoma. These look different when they are examined under the microscope in the laboratory and they affect people differently. What kind of lymphoma is nodal MZL? Nodal MZL is a low-grade B-cell non-hodgkin lymphoma that develops in the lymph nodes. Less than 2% of non-hodgkin lymphomas will be of this type, so it is a rare lymphoma. It is described as a marginal zone lymphoma because the cancerous lymphoma cells have developed from B cells that are normally seen in a part of the lymph node called the marginal zone. 2/8
What causes nodal MZL? Lymphoma develops when lymphocytes start to grow out of control. This is often due to a change within the genes of the lymphocytes. This genetic change can mean that the lymphocytes are not able to control how quickly they divide or die off. It has been noticed that marginal zone lymphomas are more common in people who have had hepatitis C virus infection and researchers think that this infection might play a part in the development of the lymphoma in a few people. It is not known why these genetic changes happen in the lymphocytes in most people with nodal MZL, however. Nothing you have done or not done will have caused the lymphoma to develop. Nodal MZL is not a disease that you can inherit or pass on to your children. What are the symptoms of nodal MZL? The most common symptom is the appearance of one or more lumps, most commonly in the neck or groin. These are enlarged lymph nodes. They are not usually painful. Often there are several lymph nodes affected, for example in the neck, armpits and groin and internally in the abdomen. Some people report that they have been feeling very tired. A few people experience weight loss, fevers and night sweats (these three symptoms are known as 'B symptoms'). What tests will I need? As well as a physical examination, you will have tests done to find out exactly what kind of lymphoma you have to make a diagnosis. Tests will also be done to check your general health and to find out whether the lymphoma is in one place or in more than one place. Assessing how much of the body is affected is called staging. Don t worry if you don t seem to be having the same tests as other people you meet who have lymphoma. The medical team will order all the tests that are necessary, depending on your symptoms and on what they find when they take your history and examine you. You are likely to have: a biopsy: a lymph node will be removed, either completely or partially, by a surgeon, usually after you have had a general anaesthetic (an excision biopsy). Sometimes a smaller sample of a lymph node is removed (a core biopsy) and this is often done under local anaesthetic. The node will be examined under a microscope and it might also be sent away for more detailed tests, sometimes to another laboratory. These tests are done to look at the types of proteins on the surface of the lymphoma cell (immunohistochemistry tests) and at the genetic make-up of the lymphoma cells (cytogenetics tests). The results of these specialised tests can take 2 3 weeks to come back. blood tests: to check for anaemia or other low blood cell counts, to check that your kidneys and liver are working well and to detect infections such as hepatitis C virus infection. 3/8
scans of your neck, chest, abdomen and pelvis. The most usual scan is computed tomography a CT scan. This kind of scan produces pictures of your internal organs in cross-section, from top to bottom, and will show up internal lymph nodes and organs. Some people will have another kind of scan called positron-emission tomography (a PET scan) if their specialist feels this would be helpful. a bone marrow biopsy, to find out if there is any lymphoma in the bone marrow. This test involves taking a sample of bone marrow through a needle. The needle is usually inserted into your hip bone after a small area of skin has been numbed using a local anaesthetic. A bone marrow biopsy takes around 15 minutes. It can be difficult to diagnose nodal MZL because it can look similar to some other kinds of lymphoma. In addition, sometimes the lymphoma or part of the lymphoma can become a faster-growing, high-grade type of lymphoma, where the lymphoma cells seen under the microscope are large. This is known as 'transformation'. Transformed nodal MZL needs to be treated differently from untransformed nodal MZL, with more intense treatments. This means that you might have to wait for some time before test results come back from specialist laboratories and sometimes tests have to be repeated. It is difficult to wait for results when you are feeling anxious, but it is important to have an accurate diagnosis so that you can be started on the most suitable treatment for your kind of lymphoma. Please ring our helpline on 0808 808 5555 if you would like to talk to someone about tests for lymphoma. We have information on tests and on transformation. You can ask the helpline to send this to you or you can download the information from our website (www.lymphomas.org.uk). What are the stages of nodal MZL? When all the test results are ready, your specialist will be able to tell what parts of your body are affected by the lymphoma. This is called the stage of your lymphoma. The following table explains what each stage means. Stage I Stage II Stage III Stage IV One group of lymph nodes is affected Two or more groups of lymph nodes are affected on one side of the diaphragm (a sheet of muscle that separates the chest from the abdomen) Lymph nodes are affected on both sides of the diaphragm Lymphoma can be found in organs outside the lymphatic system or in the bone marrow In addition to these numbers, you might also hear the letters A' or B' used to describe the stage of your lymphoma. This refers to whether or not you have had any weight loss, night sweats or fevers (the B symptoms). B means that you have had one or more of these symptoms and A means that you have not. 4/8
How is nodal MZL treated? How your lymphoma will be treated will depend on where it is and its stage. Nodal MZL is usually slow-growing and it is treated in a similar way to the most common low-grade non-hodgkin lymphoma, follicular lymphoma. Although nodal MZL is rarely considered to be curable, doctors will be aiming to get you into a long-lasting remission (when tests will show no signs of active lymphoma). Treatment of early-stage nodal MZL If you have stage IA or stage IIA nodal MZL, this is described as 'early-stage' lymphoma. For some people the best treatment at first might be a period of observation. This is called watch and wait. This might seem unexpected when you have been diagnosed with a type of cancer, but it has been shown that, for some people, monitoring the lymphoma like this makes no difference to how long you survive. In addition, delaying the treatment also delays having the side effects of treatment. If the medical team feel that the lymphoma should be treated rather than observed, the usual treatment is radiotherapy to the affected lymph nodes. Stage I nodal MZL is potentially curable, but it is unusual to have nodal MZL at this early stage. Treatment of advanced-stage nodal MZL Most people with nodal MZL have lymphoma in several places when it is diagnosed. Some people with stage II nodal MZL and anyone with stage III or IV nodal MZL will be considered to have 'advanced-stage' lymphoma. Lymphoma is not like many other cancers, however, where spread to other areas can be bad news. This is because effective treatments are available for nodal MZL at all stages. The treatments that are given for advanced nodal MZL are: single-agent chemotherapy treatment with just one chemotherapy drug, such as chlorambucil, fludarabine or cladribine combination chemotherapy treatment with several chemotherapy drugs given together, such as CVP (cyclophosphamide, vincristine and the steroid prednisolone) or CHOP (cylcophosphamide, hydroxydaunorubicin, vincristine [Oncovin ] and prednisolone) antibody treatment treatment with a specially manufactured protein antibody such as rituximab, either given alone or together with chemotherapy (eg as R-chlorambucil, R-CVP) antiviral drugs for people whose tests showed evidence of hepatitis C virus infection. Some chemotherapy drugs can affect fertility. If this is a possibility, your doctor will discuss this with you. Men might want to bank some sperm before starting treatment with these drugs. It is not common at the present time for women to store eggs but you can discuss the options that might be open to you with your medical team. 5/8
Research is being done to find other possible treatments for nodal MZL. You might be offered an opportunity to have one of these new drugs as part of a clinical trial. Follow-up after your treatment Once the treatment is finished you will be seen in the clinic every 1 3 months at first, then every 6 months after a year or so for a further 2 years. Eventually you will be seen once a year. How long you will be followed up for will depend on the treatments you had and also varies from centre to centre. Usually people are followed up for at least 2 5 years. If the specialist feels they would be helpful, you might have blood tests, X-rays and scans done during your follow-up, but they are not done routinely. What happens if nodal MZL comes back? Nodal MZL is a slow-growing lymphoma that usually responds well to treatment. It is common for this lymphoma to relapse (come back), however, and this is most likely to happen during the 2 years after your initial treatment. Nodal MZL can come back where it was before or it can come back in other lymph nodes, so you should report any new lumps or other symptoms to your medical team or your GP. Nodal MZL can occasionally relapse in the spleen or in other places in the body. If it is suspected that the lymphoma has come back your medical team will organise some more tests. It is very disappointing if the lymphoma comes back but relapsed nodal MZL can be successfully treated again. Further treatment will be planned on the basis of your individual situation, taking into account your symptoms, the results of the tests and your overall general health. Chemotherapy and other drug treatments Most people with relapsed nodal MZL will be offered further chemotherapy. Relapsed nodal MZL is also sometimes treated with one of the newer drugs that are being researched in the treatment of lymphoma. These are often only available if you are taking part in a clinical trial. If you are interested in taking part in a clinical trial, ask your specialist if there might be one suitable for you. High-dose chemotherapy and stem cell transplantation Some people with nodal MZL that has relapsed, especially if it has relapsed quickly, might be offered high-dose chemotherapy and stem cell transplantation. In this treatment, high doses of chemotherapy are given first, to kill off all the lymphoma cells. This treatment causes so much damage to your bone marrow that it might never recover by itself. Instead it is rescued (helped to recover) by stem cells. Stem cells are immature cells that are used by our bodies to replace damaged cells. Stem cells are collected from you before you have the high-dose chemotherapy and stored until you need them. The stem cells are then put back into your bloodstream, just like a blood transfusion. They then settle in your bone marrow where they start to grow and make new blood cells. This type of stem cell transplant is called an 'autologous' transplant. This is a challenging course of treatment and it is only offered to people whose general health is judged to be good enough to tolerate it. 6/8
We have separate information on watch and wait, radiotherapy, chemotherapy, antibody treatments and stem cell transplants. We also have a booklet about taking part in clinical trials. Please call the helpline if you would like to talk to someone about your treatment or if you would like this information sent to you (0808 808 5555). The information is also available on our website (www.lymphomas.org.uk). Acknowledgement We are grateful to Dr Andrew Wotherspoon, Consultant Histopathologist at The Royal Marsden Hospital, London for reviewing this information. Useful organisations Macmillan Cancer Support 0808 808 0000 (Monday Friday, 9am 8pm) www.macmillan.org.uk CancerHelp UK CancerHelp UK is the patient information section of Cancer Research UK. 0808 800 4040 (Monday Friday, 9am 5pm) www.cancerresearchuk.org Sources used in this information Our information is written using the most up-to-date published research available and current nationally recognised guidelines. If you want to know which textbooks, guidelines and research papers we used as sources for this information, please contact us by emailing the publications team on publications@lymphomas.org.uk or ring us on 01296 619409. 7/8
How we can help you We provide: a Freephone helpline providing information and emotional support 0808 808 5555 (9am 6pm Mondays Thursdays; 9am 5pm Fridays) or information@lymphomas.org.uk booklets and other information about lymphoma (free of charge) a website with forums www.lymphomas.org.uk the opportunity to be put in touch with others affected by lymphoma through our buddy scheme a nationwide network of lymphoma support groups. How you can help us We continually strive to improve our information resources for people affected by lymphoma and we would be interested in any feedback you might have. Please visit www.lymphomas.org.uk/feedback or email publications@lymphomas.org.uk if you have any comments. Alternatively please phone our helpline on 0808 808 5555. We make every effort to ensure that the information we provide is accurate but it should not be relied upon to reflect the current state of medical research, which is constantly changing. If you are concerned about your health, you should consult your doctor. The Lymphoma Association cannot accept liability for any loss or damage resulting from any inaccuracy in this information or third party information such as information on websites which we link to. Please see our website (www.lymphomas.org.uk) for more information about how we produce our information. Lymphoma Association PO Box 386, Aylesbury, Bucks, HP20 2GA Registered charity no. 1068395 Produced: January 2014 Next planned review: 2016 8/8