Low-grade non-hodgkin lymphoma (NHL)

Transcription

1 Low-grade non-hodgkin lymphoma (NHL) For adults and children with blood cancer bloodwise.org.uk Patient information

2 My details This is a place to put important information about you, your condition and key contacts. Name and hospital number My NHS number My condition My contacts My consultant My key worker (usually your CNS) Haematology ward Haematology clinic Out of hours Other contacts

3 Our patient information is for you and those close to you to use whenever, wherever and however you need it. You ll probably have lots of questions; this booklet aims to answer as many of them as possible. A team of people helped produce this booklet. We d like to thank a member of our Medical Advisory Panel, Doctor Robert Marcus, for his help and support in developing the content and checking for clinical accuracy. The draft was also assessed at an early stage by clinical nurse specialist Karen Stanley. Bloodwise staff revised the text to make it easy to read, and patients checked it for understanding. A member of Bloodwise s Medical Advisory Panel, Doctor Robert Marcus, is responsible for the content overall. Our information is developed for and with patients. It s written in line with national guidelines and created with health professionals from our dedicated Medical Advisory Panel, so you know it s accurate and up to date. This booklet is one of many we make you can find a list of our other booklets on pages For the very latest information, visit our website. Our booklets contain general information. Always listen to the advice of your specialist about your individual treatment because every person is different. When you see the symbols below in the booklet, it s a sign that we think the websites and other organisations mentioned will also give you good information and support. A list of references used in this booklet is available on request. Please us at patientinformation@bloodwise.org.uk Disclaimer We make every effort to make sure that the information in this booklet is accurate, but you shouldn t rely on it instead of a fully trained clinician. It s important to always listen to your specialist and seek advice if you have any concerns or questions about your health. Bloodwise can t accept any loss or damage resulting from any inaccuracy in this information, or from external information that we link to. The information in this booklet is correct at the time it was printed (March 2015). Date of next review March Bloodwise, Eagle Street, London WC1R 4TH T: E: info@beatbloodcancers.org.uk W: bloodwise.org.uk All rights reserved. No part of this publication can be reproduced or transmitted without permission in writing from Bloodwise. Our website Another of our booklets Registered charity (England & Wales) SC (Scotland) 26 Another page of the booklet Another organisation

4 Contents 3 Introduction 4 Low-grade NHL at a glance 7 Your lymphatic and immune system Introduction This is a booklet for people with low-grade non- Hodgkin lymphoma (NHL), and for people who know someone with low-grade NHL. It mainly talks about the most common type of low-grade NHL, follicular NHL. 11 What is low-grade NHL? 15 Who gets low-grade NHL? 19 Symptoms 23 Diagnosis 43 Treatment 55 The outlook 57 Everyday life and low-grade NHL 65 Research and new developments 69 Places you can get help and support Being told that you, or a loved one, have any type of cancer can be one of the hardest things you ll ever have to hear. There s sure to be a lot of information to take in at this time. We hope this booklet will help you to understand your condition and feel in control. We ll answer as many of the questions you may have along the way as we can from symptoms through to tests, treatment and living with lowgrade NHL, and where you can get support. Every person is different, with a different medical history. So when you re deciding what s right for you, discuss your situation with your specialist as well as getting information from this booklet and other places. 75 Questions to ask 79 Notes 83 About us 86 How you can get involved 90 Your feedback and other booklets We also produce a diary which you can order online. It s yours to use however you like for practical information or to record thoughts or sketches > bloodwise.org.uk/ patient-diary

5 4 Low-grade non-hodgkin lymphoma at a glance Low-grade non-hodgkin lymphoma at a glance 5 Low-grade non-hodgkin lymphoma at a glance In most cases, we don t know what causes low-grade non-hodgkin lymphoma (NHL). Treatment will be different for each person. As low-grade NHL is a slow growing disease, it can be controlled for many years, with some patients never even needing to have treatment. For more information on high-grade NHL, you can read our booklet > High-grade non-hodgkin lymphoma What is low-grade NHL? Low-grade non-hodgkin lymphoma (NHL) is a blood cancer that affects a type of white blood cell called a lymphocyte. There are two main types of NHL, which are grouped depending on how quickly the cancer grows and spreads. The two types are high-grade lymphoma, which develops quickly; and low-grade lymphoma, which develops more slowly. If you have low-grade NHL, your cancer will also be defined by your symptoms and by how much lymphoma there is in your body. This is known as the stage of the cancer, which will determine the treatment you ll be offered. Who gets low-grade NHL? In most cases we don t know what causes low-grade NHL. It s more common in older people, and it s rare in children. There are some factors, like age, gender and having particular viruses, which can affect how likely you are to get lowgrade NHL. What are the treatments for low-grade NHL? The treatment you have for low-grade NHL depends on what stage your lowgrade NHL is, and what symptoms you have when you re diagnosed. Some people with low-grade NHL might not need to have treatment. You may just have regular check-ups and careful monitoring of your condition this is called watch and wait. If you do need to have treatment, it can involve chemo-immunotherapy (anti-cancer drugs) and radiotherapy (radiation treatment). If you don t respond to your treatment or if you relapse, you ll have more treatment, which could involve more chemotherapy or a stem cell transplant. What s the outlook? As low-grade NHL is a slow growing disease, it can be controlled for many years, with some patients never even needing to have treatment. Most patients who do need to have treatment will achieve a good first remission (where there s no sign of the lymphoma in your body). There is a chance that you could relapse (the low-grade NHL comes back), but second-line treatments are available in this case. Can low-grade NHL lead to any other conditions? In some people low-grade NHL can change in nature and you could develop high-grade NHL.

6 6 Your lymphatic and immune system Your lymphatic and immune system 7 Knowing the basics about the immune system and your lymphatic system is useful. Your lymphatic and immune system It s a good idea to know a bit about the immune system and your lymphatic system as your healthcare team will talk about them to you. Non-Hodgkin lymphoma is a disease which affects your lymphatic system. The lymphatic system is part of your body s natural defence against infection, which is known as the immune system. Within your lymphatic system there s a network of thin tubes called lymph vessels which run around your body. The vessels collect fluid called lymph and return it to your blood. Lymph bathes all the cells in your body. It contains lots of lymphocytes (a type of white blood cell that fights infection), which carry nutrients and remove bacteria from infected areas. You can find out more about your lymphatic and immune system on our website > bloodwise.org.uk/nhllinks

7 8 Your lymphatic and immune system Your lymphatic and immune system 9 Along the lymph vessels are small lumps of tissue called lymph nodes or lymph glands. There are many of these in your body. It may be possible for you to feel normal lymph nodes in the neck and groin, particularly if you re slim. If you get an infection when you re healthy, these can swell and become tender, which people may refer to as swollen glands. As lymphocytes pass through the lymph nodes, they are changed and activated to fight certain types of infection. Your spleen is also part of your lymphatic system. It can do some of the same work as the lymph nodes. It also filters out old or damaged cells from the blood stream and helps to fight infection. Your immune system Your immune system is a network of cells, tissues and organs which protect your body against infection. It s able to react quickly to infections it s seen before, and lymphocytes play an important role in this. There are lots of different kinds of lymphocyte, including ones called T cells and B cells. These can be affected when you have lymphoma, which can increase the risk of infections. Your healthcare team can let you know about the ways to reduce your risk. Lymphatic system Barriers nose, mucous Thymus Tonsils Lymph nodes (neck) Lymphatic vessels Lymph nodes (armpit) Spleen Bone marrow Lymph nodes (groin) You can also find more information about how to manage infections in our booklet > Supportive care Lymphatic vessels

8 10 What is low-grade NHL? What is low-grade NHL? 11 There are many different types of lymphoma. Make sure to check with your specialist that this is the right booklet for you. What is low-grade NHL? Lymphoma is a cancer of the lymphatic system. Non-Hodgkin lymphoma is a type of lymphoma which usually affects B cells. There are different types of non-hodgkin lymphoma, which are grouped depending on how fast the lymphoma grows and spreads. Lymphoma Lymphoma is a cancer of the lymphatic system. When you have lymphoma some of your lymphocytes don t work properly. Sometimes they aren t developed fully (they re immature), they divide in an abnormal way, or don t die when they should. These abnormal lymphocytes can build up in your lymph nodes, causing them to swell and form a lump. Swollen lymph nodes can be in a place where they can be easily felt (such as your armpits, neck or groin) or they can be deep inside your chest or abdomen. Lymphoma can occur anywhere in the body where lymphocytes collect. The abnormal lymphocytes can affect how your immune system works, which can sometimes mean you re more likely to get infections.

9 12 What is low-grade NHL? What is low-grade NHL? 13 Non-Hodgkin lymphoma NHL is divided into two main groups: Low-grade lymphoma: this usually develops slowly and sometimes doesn t require treatment at the time of diagnosis. High-grade lymphoma: this usually develops more quickly and usually requires more urgent treatment. This booklet mainly talks about the most common type of low-grade NHL called follicular lymphoma. This is a type of low-grade NHL which affects B cells. There are some other types of low-grade NHL which behave, and are treated differently to follicular lymphoma. If you have one of the following types, please see our signposts below to a separate booklet or fact sheet, or another organisation. As each individual case is so different, particularly in non- Hodgkin lymphoma, your consultant is the best person to ask about your type of lymphoma and your treatment. Ask your doctor if you re not sure which booklet is right for you. waldenström macroglobulinaemia (WM), also referred to as Lymphoplasmacytic lymphoma (LPL), is a disorder with similarities to NHL and myeloma small lymphocytic lymphoma (SLL), which is very similar (if not identical) to chronic lymphocytic leukaemia marginal zone lymphomas cutaneous lymphomas mantle cell lymphoma is a type of lymphoma that falls between lowgrade and high-grade; treatment will be different to the standard low-grade NHL treatment. For more information on WM, read our factsheet > Waldenström macroglobulinaemia (WM) If you ve been diagnosed with SLL, please see our booklet on chronic lymphocytic leukaemia > Chronic lymphocytic leukaemia For more information on marginal zone lymphomas, cutaneons lymphomas and mantle cell lymphoma, please visit the Lymphoma Association website > go to lymphomas.org.uk then search for the disease name.

10 14 Who gets low-grade NHL? Who gets low-grade NHL? 15 You re not alone. About 3,000 people are diagnosed with low-grade NHL every year in the UK. Who gets low-grade NHL? When you re diagnosed with any cancer, one of the first things you might think is: why me? In most cases, we can t say what causes low-grade NHL. However, there are some factors that could make you more likely to develop low-grade NHL. How common is NHL? Around 10,000 people are diagnosed with a type of non- Hodgkin lymphoma every year in the UK. About one-third of people diagnosed with NHL will get the low-grade type of the disease. The majority of those with low-grade NHL have a type called follicular lymphoma.

11 16 Who gets low-grade NHL? Who gets low-grade NHL? 17 When I was first diagnosed why me? was one of the first things that went through my head. Then I wanted to know what caused it, but with low-grade NHL it s not something you can point to. Age Family history Infections You can get low-grade NHL at any age, but it mainly affects older people. The average age of someone diagnosed with NHL is about 65. It s uncommon for people under 40 to get low-grade NHL. Low-grade NHL is very rare in children. Gender Men are slightly more likely than women to develop low-grade NHL; we don t know why. In most cases, low-grade NHL doesn t run in families. However, rarely, in some families there are more cases of low-grade NHL than would be expected to occur just by chance. If you feel this is the case for your family, please do discuss it with your doctor. You might have a higher risk of getting NHL if you ve had some viral infections, including the HIV virus and human T-cell leukaemia/lymphoma virus (HTLV-1). The HTLV-1 virus most commonly occurs in patients of Japanese or Afro-Caribbean origin. The Epstein-Barr virus (EBV), which causes glandular fever, can sometimes lead to NHL. This virus is common, but only a very small number of people who get the virus go on to develop NHL and this is more usually related to more aggressive types of lymphoma. It s important to remember that although these viruses can be passed from person to person, you can t catch lymphoma from someone else. And only a tiny number of people will ever then go on to develop NHL; a lot of things have to happen after you get the virus, for it to turn into NHL. For more information on the causes of low-grade NHL, see our website > bloodwise.org.uk/nhllinks

12 18 Symptoms Symptoms 19 If you get new symptoms after you ve been diagnosed, or if you feel unwell, contact your hospital as soon as possible. Symptoms Some people who get low-grade NHL will have some of the symptoms listed below. It s important to remember that not everyone will get all of these symptoms. Main symptoms The main symptom of low-grade NHL is having swollen lymph nodes, which causes a lump. You might have heard this being called swollen glands. Your glands will get bigger quite quickly but won t necessarily be painful. The most common place for you to notice these would be in your neck, armpit or groin. In these areas the lymph nodes lie just under the skin, so you re more likely to notice if they re swollen.

13 20 Symptoms Symptoms 21 Sometimes, the affected lymph nodes can be deeper in your body. You may not be able to see the swelling, but you might have other symptoms caused by this. The symptoms you get will depend on where the swollen lymph nodes are in your body, and which organs they re pressing against. These symptoms might include: chest or abdominal (stomach area) pain bone pain skin lumps coughing or breathlessness. Other symptoms You might also have some of the following symptoms. Together these are known as B symptoms : fever (greater than 38 C) drenching night sweats which soak your nightclothes and bedding (although this symptom is more common in high-grade non-hodgkin lymphoma) unexplained weight loss in the last six months (10% or more of your previous weight). If you have low-grade NHL, you may also have fatigue this is when you feel tired much of the time. It s not the same as ordinary tiredness which improves with sleep or rest. Once I d been diagnosed, I realised that I d had a lot of the common symptoms of NHL, but I hadn t joined the dots before. You can find out more about symptoms of NHL on our website > bloodwise.org.uk/nhllinks

14 22 Diagnosis Diagnosis 23 It s important to know and understand your diagnosis. You could ask your team to write it in this booklet, so you have it to hand. Diagnosis You ll have a number of tests and scans to confirm whether you have low-grade NHL. These will also show how much the lymphoma has grown and developed doctors call this staging the lymphoma, or they ll refer to the stage of the condition. Doctors will also check your general health. Sometimes, you might need to prepare before a test. This might include fasting (having nothing to eat or drink except water). Your doctor will tell you what each test will involve and if you need to prepare in any way. At any time, you can ask your healthcare team to tell you why you re having a certain test and what the results mean.

15 24 Diagnosis Diagnosis 25 Tests to diagnose low-grade NHL The only way to confirm a diagnosis of low-grade NHL is through a lymph node biopsy. Lymph node biopsy A lymph node biopsy is a minor surgical procedure that takes a sample of the lymph node affected by the cancer. This sample is then examined under the microscope in a laboratory. This test may be done under general anaesthetic. You ll often have one whole lymph node removed you might hear this being called an excision biopsy. Increasingly a core of lymph node is removed under local anaesthetic with the help of an ultrasound or CT scan. This is called a core biopsy or incisional biopsy. This is the definitive test that will confirm your diagnosis of lymphoma. After this test, your lymphoma will be staged to understand the extent of the cancer. Initial health tests After your diagnosis, you ll have more tests which will give doctors more information about your health and the lymphoma. Blood tests These will give your healthcare team a full picture of your general health before you start any treatment. Full blood count A full blood count (FBC) measures the number of each type of cell in the blood: red cells, white cells and platelets. Although the full blood count is often not affected in low-grade NHL, the test is still important. The FBC will help doctors spot if you develop anaemia (a condition caused by a lack of red blood cells which can lead to tiredness and breathlessness). Anaemia may occur because of your illness or your treatment. The FBC will also see if the lymphoma has affected your bone marrow and if this happens your treatment might be changed. Liver function tests This test checks if your liver is working normally. This is important to know about if you re having chemotherapy because a lot of drugs are broken down in the liver. If your liver isn t working normally, your doctor might need to adjust the doses of your treatment drugs. Urea and electrolytes This test checks how well your kidneys are working. This will help decide the doses of the drugs you ll need in your treatment. This test will also show if you ve become dehydrated, or if the cancer or the treatment has caused any damage to your kidneys. You ll usually have your kidney function checked every time you have an appointment at the hospital. Infection screening It s now normal practice for you to be tested for some viruses which can cause infections. If you have low-grade NHL, your immune system isn t working properly, so these viruses may cause serious acute disease. You ll be tested for hepatitis B, hepatitis C and HIV/AIDS. Your test results might determine which treatments you have if this is the case, your doctor will discuss this with you. Cytogenetics It s becoming more common for doctors to use special investigations to find out how developed the cancer is. These include molecular cytogenetics and gene sequencing tests. The test will be done in the lab using your bone marrow sample. These tests look at the genetic abnormalities in the lymphoma cells, which might help determine the treatment you have.

16 26 Diagnosis Diagnosis 27 Staging, grading and risk scoring When you re first diagnosed, your specialist will carry out tests to allow them to stage, grade and risk score the lymphoma. Staging Your doctors will want to know the stage of your lymphoma. Staging describes how far the lymphoma has spread and the nature of the lymphoma. The stage of your lymphoma will affect your doctor s recommendation about whether you should start treatment immediately and which treatment is right for you. There s a standard system which all doctors use to stage low-grade NHL. It s mainly based on how many areas of your body are affected by the lymphoma. The staging groups are shown in the table opposite. Staging is also based on which symptoms you have when you re diagnosed. This is shown by a letter A or B any letter can apply to any of the numbered stages on the opposite page. The most important distinction in staging is between disease which hasn t spread, called early disease (stage I or II disease with no B symptoms), and advanced disease (all other cases). This distinction is important because there are different treatment options for early stage and advanced stage disease. Grading Grading is a specific term which is only used to describe follicular NHL. Follicular NHL is always classed as a low-grade lymphoma, and given a stage accordingly. However, as well as staging, follicular lymphoma is also given a grade from 1 to 3 (with an A or B, as described in the table opposite). Grading of follicular NHL looks at types of cells called centroblasts (a type of B cell). Grading looks at the affected node, and the proportion of centroblast cells compared with other cells. These grades can sometimes help doctors see how fast the lymphoma is growing. Groups 1, 2 and 3A are classed as follicular NHL. If you have grade 3B follicular NHL, your condition is likely to be treated as a high-grade lymphoma. STAGE I (1) II (2) III (3) IV (4) A B FEATURES Only one group of lymph nodes is affected, in one place in your body. More than one group of nodes is affected but all affected sites are on the same side of the diaphragm either above or below. The diaphragm is a sheet of muscle separating your chest from your abdomen (stomach area) and pelvis (hip area). Lymph nodes on both sides of the diaphragm are affected, or the lymphoma has spread from lymph nodes into organs close to the affected node, or nodes. The lymphoma has spread to other organs, such as the lungs, liver or bone marrow. SYMPTOMS No B symptoms (see below) B symptoms are present: fever (greater than 38 C) drenching night sweats which soak your nightclothes and bedding unexplained weight loss in the last six months (10% or more of your previous weight).

17 28 Diagnosis Diagnosis 29 Risk scoring Risk scoring helps your doctor predict how your condition will respond to treatment. There are a number of guidelines your doctor will use to predict how you re likely to respond to treatment. These are called the follicular lymphoma indices. They look at several different aspects of your disease, including but not limited to: your age stage which lymph nodes are involved your haemoglobin levels (the levels of iron in your red blood cells) your LDH levels (the levels of a biochemical called lactate dehydrogenase in your blood) the size of the largest mass of cancer cells (swollen node/s). You ll have a series of tests which will help your doctors to find out the stage of the low-grade NHL. These tests will also help doctors predict how you ll respond to treatment. You ll have heard of some of these tests, but others might be less familiar. You can always talk to your healthcare team if you have any questions. Staging tests Bone marrow aspirate and trephine You may have a bone marrow biopsy (sample) taken soon after diagnosis, to see whether the bone marrow is affected by lymphoma. This may give doctors another opportunity to confirm your diagnosis and gain extra information about the disease. During the procedure a small amount of bone marrow is sucked from the hip bone using a fine needle (an aspirate). Your doctors will then look at the bone marrow sample under a microscope. You don t need to stay overnight in hospital for a bone marrow biopsy; you can have it as an outpatient using local anaesthetic or entonox (gas and air). It s usually quite quick but will be uncomfortable while the sample is being taken from your marrow; you can take painkillers if you need to. You ll also have a bone marrow trephine. This is taken at the same time as the bone marrow aspirate, and a slightly larger piece of bone marrow is taken from the same site. You might experience some discomfort around the area where your bone marrow sample was taken. If you d like, you can take some paracetamol to help with the pain. Any discomfort is usually gone after hours. For more information on staging, grading and risk scores for low-grade NHL see our website > bloodwise.org.uk/ NHLlinks

18 30 Diagnosis Diagnosis 31 Scans Doctors will use scans to help with your diagnosis and staging. These often use a form of radiation, but your healthcare team will always make sure that you re not exposed to more radiation than is absolutely necessary. It may take a few weeks to get the results of all these tests, which can be a worrying time for many people. It s really important that doctors take the time to get an accurate diagnosis and stage the lymphoma correctly, as this will determine what treatment you have. X-rays X-rays provide good images of the denser tissues in your body, such as bone. You might have more x-rays during your treatment, to find out how your body is responding and to check for chest infections. You can find out more about the diagnosis of low-grade NHL, and other topics covered in this booklet, on our website > bloodwise.org.uk/nhllinks

19 32 Diagnosis Diagnosis 33 CT scan CT scans look at your soft tissues (the non-bony parts of your body). It s likely that you ll have a full body CT scan, which gives a detailed picture of your internal organs. The scan isn t painful. You ll lie on a table that moves into a cylindrical tunnel while the pictures are taken. Your body is never completely enclosed and you ll be able to talk to the person who takes and assesses the images (the radiographer) all the time. You might need to have a dye injected into one of your veins, to help get a better image. If you re feeling anxious about this procedure, you may be given a sedative to take beforehand. Fluorodeoxyglucose positron emission tomography (FDG-PET scan, PET scan or PET/CT scan) You ll almost always have a PET scan (or sometimes a combination scan called a PET/CT scan) to help stage the cancer. PET scans are often combined with CT scans. For this scan, you ll have an injection that contains radioactive sugar. This small amount of radioactivity is completely safe. Like with CT scans, you ll lie on a table as you move through a cylindrical tunnel. The scan can take up to three hours, including preparation time. You ll get more information and advice about this scan from your hospital. As the cancerous cells take up more of the radioactive sugar than noncancerous cells, doctors will be able to measure how much lymphoma is in your body. You may have this scan again halfway through or at the end of your treatment to see if it s been successful.

20 34 Diagnosis Diagnosis 35 Magnetic resonance imaging (MRI) An MRI uses the effect of a strong magnet on your body to produce very detailed images of soft tissues, which are then analysed on a computer. During the test, you ll lie on a table which will move you through the scanner. To help the computer create a better picture, you might need to have some fluid injected into a vein using a needle. It ll take around an hour to carry out the scan. It s not painful but the MRI machine is noisy you might be given headphones so you can listen to music during the scan. Ultrasound scans Ultrasound scans can be used to give more information on your condition. For example, it could be used to measure the size of your spleen, which is often enlarged in lymphoma patients. I did feel from time to time that I couldn t keep track of all the tests and what they were for. I often wrote down basic details, and just kept checking in with my key worker on the specifics for each one. Special tests Your doctors will also do several other tests on your blood and/or bone marrow samples. These will help predict how the low-grade NHL might respond to treatment. These are known as prognostic markers. Serum β2- microglobulin (Sβ2-M) You ll have a test that looks at the level of a protein called serum β2- microglobulin (Sβ2- M) in your body. Some patients with NHL will have higher amounts of Sβ2-M than is normal, and this can tell doctors how advanced the low-grade NHL might be. Lactate dehydrogenase (LDH) When cells break down, they release a chemical called lactate dehydrogenase (LDH). If you have low-grade NHL, you ll normally have more LDH in your body because more cells are being produced and more cells are dying. This test can tell doctors how advanced the low-grade NHL might be.

21 36 Diagnosis Diagnosis 37 For a list of support organisations > see page You can read about the experiences of other people who are going through or have been through the same thing on our website > bloodwise.org.uk/patient-support Your healthcare team If you re diagnosed with low-grade NHL, your hospital will give you the names and contact details of your consultant, clinical nurse specialist and other members of your healthcare team there s space to write them at the back of this booklet if you want to. You can then use these details to contact your team if you have any questions you want to ask when you re not in the hospital. Your consultant Most patients with a blood cancer are treated by a haematologist a doctor who specialises in treating patients with blood diseases like blood cancer. Some patients are treated by an oncologist a cancer specialist. Either way, your consultant at the hospital will be an expert in treating your specific disease. Your clinical nurse specialist All cancer patients are given a key worker, usually a clinical nurse specialist. They are your point of contact with the rest of your healthcare team. You may like to have a meeting with your clinical nurse specialist when you re first diagnosed, to discuss your condition. Really make use of your clinical nurse specialist as they ll be with you right through your cancer journey. Your multidisciplinary team When you re diagnosed with something like low-grade NHL, your condition is discussed at a multidisciplinary team (MDT) meeting. An MDT brings together doctors, nurses and any other specialist staff who will be looking after you. A senior consultant usually leads the meetings, which are held regularly. They ll discuss the best treatment for you and every aspect of your care, including any changes in your condition. Talking to other patients You might want to ask your consultant or key worker if you can talk to someone who s had the same diagnosis and treatment as you. If you do this, remember that someone else s experience won t always be the same as yours. For example, some patients have side effects from a drug and other patients don t. You may also want to contact a support organisation many provide patient meetings or further online support. Your other healthcare professionals It s definitely worth telling other healthcare professionals you see like your dentist or optician about your diagnosis and any medication you re taking.

22 38 Diagnosis Diagnosis 39 Finding out more After you ve been diagnosed, it s worth taking some time to think about what information you want to know, when and how. For some people, this is a way to have some control over what s happening. Let your consultant and clinical nurse specialist know how much information you d like, and in what form. You can always ask for more information later. Write down any questions you have and keep them handy for when you see your consultant or key worker. If they can t answer your questions, they ll be able to tell you who to speak to. You might prefer to ask your clinical nurse specialist questions rather than your consultant, but do whatever works for you. Most patients say they find it useful taking someone with them to consultations. If you d find it helpful, you could ask them to take notes while you listen. You can choose who to take; it doesn t have to be a family member. If you re staying in hospital it might be harder to have someone with you when you speak to your consultant. It might be useful to ask in advance what time the consultant is likely to speak to you, so you can try to arrange for someone to be with you at that time. Some people find that joining a patient support group is helpful. It may be easier to talk to someone outside of your family about your situation and being able to share similar experiences might also help you. Your questions 75, 79 You can find a list of questions you might want to ask on page 75 and room to write more questions on page 79

23 40 Diagnosis Diagnosis 41 I didn t know whether to tell people I had cancer and at first only told people on a need-to-know basis after all, I looked well physically so didn t think people would understand. But I ve found since telling people that I ve largely continued to be treated as me rather than me with cancer. Macmillan make a booklet about talking to children about cancer > go to macmillan.org.uk then search for talking to children and teenagers when an adult has cancer Telling people Many patients tell us that keeping in touch with loved ones throughout their illness keeps them going. However, some people may find it stressful having to discuss their condition lots of times with family, friends and colleagues. You might find it easier to ask a trusted family member or friend to be your information person and ask them to keep people updated on your behalf. Another idea is setting up a blog or Facebook page, so you or different people can post information on it that everyone can read. You might not want to tell many people or anyone at all about your condition. This is ok too, whatever works for you. Telling children and teenagers Talking to children and teenagers about your cancer diagnosis can be difficult. There are lots of organisations that are able to support you and offer you advice about how to explain cancer to children of different ages. Telling your GP Your team at the hospital will keep your GP informed about your condition and any treatment you re having. They ll usually send your GP a letter with this information. As the patient, you ll often be sent a copy too. These letters can have a lot of medical terms in them which you might not have heard before, or there might be something in it which worries you. If this is the case, let your hospital or GP know a quick chat with them might help to reassure you. Cancer and work Consider telling someone at work about your diagnosis. It can be hard asking for time off at short notice if no one knows about your illness, and your colleagues and human resources department might be able to offer support. Macmillan have some useful advice about cancer and work online; you can also order a booklet > go to macmillan.org.uk then search for work 62 There s more information about cancer and how it can affect your work or study on page 62

24 42 Treatment Treatment 43 The treatment you decide on with your healthcare team will depend on your health, your individual condition and your wishes. Treatment The treatment that s best for you will depend on the stage of the low-grade NHL and your symptoms. Your healthcare team will always discuss your treatment options with you, and explain why a certain treatment has been recommended. Your wishes will be taken into account when planning your treatment. You can choose not to have treatment, even if your doctor recommends it, but you can t insist on starting treatment if your doctor doesn t think that it s in your best interest. If you have any concerns about treatment you should discuss these with your consultant or clinical nurse specialist before making a decision. For information about making treatment decisions, see our booklet > Treatment decisions

25 44 Treatment Treatment 45 Aims of treatment for low-grade NHL It s rare that we can say that a person s low-grade NHL has been cured. However, it can be controlled for many years, and people can survive for many decades after their diagnosis. The aim of your treatment will be to get as good a response as possible, in terms of shrinking the tumour, with the fewest side effects. Some people might not need to have treatment straight away, or they might not need to have treatment for some years. This happens to around half of patients most will need to have treatment within two or three years. If you don t need to start treatment right away, you may feel anxious and this is completely understandable. It s important to understand that being on watch and wait won t alter how effective your treatment is once you start to have symptoms. It s important to remember that there s no harm or disadvantage in delaying treatment if you feel well. Risk of infertility with treatment If you need to have treatment for lowgrade NHL, and you re thinking about having children, or considering having them at some point in the future, it would be good to discuss the risk to your fertility and your options to protect this with your healthcare team before you begin treatment. For men, there can be a high risk of infertility if you have treatment. A common option for men is to have sperm stored. For women, there s a low risk of infertility if you have treatment. You might be able to have your eggs frozen or have embryo cryopreservation if you have a partner. If you re at an immediately premenopausal age, then there s a risk that having treatment may start the menopause earlier. Clinical trials If there s a clinical trial (study) available that s suitable for you, your consultant may recommend that you consider this. Clinical trials are done for several reasons, including to look for new treatment options and to improve existing treatments. Taking part in a clinical trial has many advantages, such as the opportunity to have the newest available treatment which may not be given outside of the trial. You ll also be very closely monitored and have detailed follow-up. In a clinical trial, the best current treatment is compared to one that could be better. You ll still get normal treatment while you re taking part in the trial, and your safety and wellbeing is always the first priority. Taking part in a clinical trial does come with uncertainties, and you may prefer not to take part in one. If you don t want to be in a trial, or there isn t a suitable trial available, you ll be offered the best treatment available at that time which is suitable for your individual condition. For more information, read our booklet > Clinical trials You can find out more about clinical trials in the UK Clinical Trials Gateway >

26 46 Treatment Treatment 47 Types of treatment What type of treatment you have depends on the stage of the lymphoma. Please note that stage 1B and 2B could be classed as early stage but these are rarely seen in follicular lymphoma. Early stage: stages 1A and 2A without symptoms NHL at this stage is classified as early stage this means that the lymphoma hasn t spread and you ll have no symptoms aside from a lump at the time of your diagnosis. It s also called localised lymphoma, which means that the glands affected by the cancer are close together. Only a small number of patients will fit this description when they re diagnosed. Your healthcare team will normally recommend having only localised radiotherapy (radiotherapy that s only given directly to the area where the lump is). This is usually given over about days. If after you have radiotherapy your healthcare team is confident that all the lymphoma cells have been killed, you might not need any more treatment. You ll be monitored carefully. If the radiotherapy hasn t killed all the lymphoma cells, you and your healthcare team will discuss an alternative treatment plan with you. Advanced stage with no symptoms If the lymphoma has spread but you don t have symptoms when you re diagnosed, your consultant might recommend that you don t start treatment immediately this is called watch and wait. Instead, you ll have regular appointments with your healthcare team and you ll be carefully monitored. In the first year after your diagnosis you ll usually have appointments every three months. In the second year after your diagnosis, you ll have appointments every three to four months. After two years, there ll then be bigger intervals between your appointments depending on if the disease is progressing. You ll have more scans that will look at glands inside your body, to find out if this is the case. It can be hard to find out that you have cancer but that it won t be treated immediately. If you have any questions or worries, do discuss them with your healthcare team. Our online community is also a good place to hear from other people going through the same thing. For more information on being on watch and wait, read our booklet > Watch and wait: monitoring while treatment isn t necessary You can read about the experiences of other people who are going through or have been through through the same thing on our website > bloodwise.org.uk/patient-support

27 48 Treatment Treatment 49 Remission Advanced stage with symptoms (or signs of progression) If the lymphoma has spread and you have symptoms when you re diagnosed, your initial treatment is likely to involve immuno-chemotherapy. Immuno-chemotherapy involves chemotherapy drugs given alongside an antibody treatment. During immuno-chemotherapy you ll take a combination of intravenous (given into a vein) chemotherapy drugs. This will either be with CVP, which stands for cyclophosphamide, vincristine and prednisolone (a steroid), or with a drug called bendamustine. With both of these options, you ll also receive rituximab. Rituximab is a biological therapy, which identifies a protein on the surface of the B cells which are affected by the lymphoma and kills them. Rituximab is an important part of nearly all treatment options for people with NHL. At the moment, you take rituximab as an infusion (a drip into the vein), but in the future this may be replaced by subcutaneous (under the skin) injections. Maintenance therapy following treatment The aim of maintenance therapy is to delay the return of the low-grade NHL, and it s now generally recommended as part of routine treatment. For your maintenance therapy you ll receive rituximab over the course of two years. After you have finished your treatment with chemotherapy, you ll go into the hospital every two months to have your maintenance treatment. This will consolidate your treatment and should give you longer in remission the average time to be in remission for is six or seven years. Your healthcare team will measure your body s response to your initial treatment. The result of your treatment will be described to you in one of four ways; this way of measuring remission can help your doctors decide if you need any more treatment: Complete remission when all the lymphoma has gone and there s no detectable evidence of lymphoma on your scans. Partial remission a clear reduction in the amount of lymphoma (with more than 50% of the lymphoma being killed) but it s still detectable on scans. Stable disease a reduction in the amount of lymphoma (with less than 50% of the lymphoma being killed) but it s still detectable on scans. Progressive disease when the lymphoma has grown. Most patients will have a good response to their initial treatment and will achieve a complete or partial remission. Some older and frailer patients who can t tolerate chemotherapy may receive rituximab alone, which can be effective.

28 50 Treatment Treatment 51 Relapse Unfortunately, even in cases of complete remission, the low-grade NHL will come back. This is called a relapse. If you ve relapsed, your treatment will depend on how long it s been since you were diagnosed. The longer you have been in remission before you relapse, the better your outlook is likely to be. Intermediate relapse If the disease comes back less than five years after your first diagnosis, you ll have several treatment options. They will usually include further chemotherapy. Your healthcare team may also want to discuss the option of following this with a stem cell transplant. Late relapse If the disease comes back more than five years after your first diagnosis, you may be able to have very similar treatment to what you ve already had. You may also take some trial drugs as part of your treatment. Refractory disease Sometimes, the lymphoma will not respond to initial treatment and the patient won t achieve a remission. This is called refractory disease. Refractory NHL is quite rare less than 20% of patients will get refractory NHL. In this case, you ll have experimental therapies, like trial drugs, and you may have a stem cell transplant. Treatment of relapsed and refractory low-grade NHL Trial drugs If you take part in a clinical trial for low-grade NHL, doctors will typically be testing a novel agent (or new medicine) in combination with existing drugs which are used to treat the disease. There are a number of studies looking at the effectiveness of these trial drugs, which include: idelalisib ibrutinib antibody-drug conjugates (ADCs). These are low-toxicity drugs. This means that they have fewer side effects than other drugs. At the moment these drugs are only available if you enter a clinical trial. However, you ll be able to discuss your options with your healthcare team, and if they think that these trial drugs are appropriate for you, you may be able to join one of these trials. Stem cell transplant For some patients, an autologous stem cell transplant might be an option. This is a stem cell transplant using your own stem cells. You ll have high-dose chemotherapy, sometimes combined with radiotherapy, followed by a transplant with your own stem cells. A stem cell transplant isn t a routine option for people with low-grade NHL. They re usually only recommended as an option for patients who have had an early relapse, and are medically fit enough to have this type of intensive treatment. This is because this treatment brings more side effects than other treatment options. An allogeneic stem cell transplant (a transplant using donor cells) may offer a chance of a cure. This is only an option for younger, fitter patients. If one of these stem cell transplants is an option for you, your healthcare team will discuss the potential benefits and risks of this with you before you make a decision. For more information about stem cell transplants, read our booklet > Bone marrow and stem cell transplantation (BMT)

29 52 Treatment Treatment 53 Side effects Most people who have low-grade NHL won t have major side effects. Often people can tolerate the treatment quite well. If you do have some side effects from your treatment, they re usually temporary and often go away when you ve finished your treatment. Possible side effects of treatment for low-grade NHL include: achy feelings diarrhoea skin rash constipation extreme tiredness hair loss (alopecia) infections fever bruising risk of bleeding shortness of breath mouth sores nausea (feeling sick) upset stomach and vomiting poor appetite (loss of taste) weight changes infertility. If you have any side effects from your treatment, let your healthcare team know. Often they ll be able to help you manage them for example, if you re feeling sick, you ll be prescribed antinausea drugs. Supportive care You might need supportive care alongside your other treatment. Supportive care involves dealing with complications of your illness or your treatment. This includes things such as dealing with nausea, preventing gout, avoiding and treating constipation, mouth care, blood transfusions, pain management and diet planning. Transformation of lowgrade NHL There s a possibility that low-grade lymphoma may change its nature and become a faster-growing lymphoma called high-grade NHL. Doctors can check this through a lymph node biopsy. This is called transformation, and can happen in around 30% of patients. If this is the case, you ll be treated as someone with high-grade NHL Follow-up Your follow-up arrangements will depend on what type of treatment you have and how well the cancer responds to treatment. Your healthcare team will explain how often you should come for follow-up checks. It s very important that you don t miss these appointments. A normal pattern for patients with low-grade NHL is to have periods of remission followed by relapses, so your doctor will need to monitor you in case this happens. It s also important to report any new signs or symptoms, like swollen glands, to your healthcare team, so that they can investigate these. If you ve responded well to treatment, it might be many months, or even years, before you need any more treatment. For more information on supportive care, see our booklet > Supportive care For more information on high-grade NHL, see our booklet > High-grade non-hodgkin lymphoma 24 For more information on lymph node biopsies, see > page 24