Essential Thrombocythaemia (ET)

A Guide for Patients Introduction

Being diagnosed with essential thrombocythaemia (ET) can be upsetting, particularly when you may never have heard of it before, and may even have had no obvious symptoms. If you have questions about ET – what causes it, who it affects, how it affects your body, what symptoms to expect and likely treatments – this booklet covers the basics for you.

You’ll also find useful advice reviewed by Manos Nikolousis, about how to get the best from Consultant Haematologist at your haematologist, plus practical Heart of England NHS Trust. The advice on how to help important booklet has then been updated people in your life understand by our Patient Information Writer, such a rare condition. For Isabelle Leach, and reviewed by Dr. more information, talk to your Mallika Sekhar, UCLH. We are also haematologist or clinical nurse grateful to Linda Phyall, Sabine specialist. Lurcook, Rachel Worzencraft, Sue Law, Dr Dick Morris, Paula This booklet was originally written Davis, Jeanette Bennett, Fiona and subsequently revised by Ken Bridge, Claire Todd, Cath Owens Campbell MSc (Clinical Oncology) and Lucy Geering for their and the review was conducted by valuable contributions as patient Lisa Lovelidge. It was then peer reviewers.

If you would like any information on the sources used for this booklet, please email [email protected] for a list of references.

Version 4 Printed: 07/2020 2 www.leukaemiacare.org.uk Review date: 07/2022 In this booklet

Introduction 2

In this booklet 3

About Leukaemia Care 4

What is essential thrombocythaemia (ET)? 6

Symptoms of ET 8

How is ET diagnosed? 10

What is the prognosis of ET? 11

Treating ET 12

Living with ET 16

Talking about ET 20

Glossary 23

Useful contacts and further support 27

Helpline freephone 08088 010 444 3 About Leukaemia Care

Leukaemia Care is a national charity dedicated to ensuring that people affected by blood cancer have access to the right information, advice and support.

Our services has been affected by a blood cancer. A full list of titles – both Helpline disease specific and general Our helpline is available 8:30am information titles – can be – 5:00pm Monday - Friday and found on our website at www. 7:00pm – 10:00pm on Thursdays leukaemiacare.org.uk/support- and Fridays. If you need someone and-information/help-and- to talk to, call 08088 010 444. resources/information-booklets/

Alternatively, you can send Support Groups a message via WhatsApp on Our nationwide support groups 07500068065 on weekdays are a chance to meet and talk 9:00am – 5:00pm. to other people who are going Nurse service through a similar experience. For more information about a We have two trained nurses on support group local to your area, hand to answer your questions go to www.leukaemiacare.org. and offer advice and support, uk/support-and-information/ whether it be through emailing support-for-you/find-a-support- [email protected] or group/ over the phone on 08088 010 444. Buddy Support Patient Information Booklets We offer one-to-one phone We have a number of patient support with volunteers who have information booklets like had blood cancer themselves this available to anyone who or been affected by it in some

4 www.leukaemiacare.org.uk way. You can speak to someone Website who knows what you are going You can access up-to-date through. For more information information on our website, on how to get a buddy call www.leukaemiacare.org.uk. 08088 010 444 or email [email protected] Campaigning and Advocacy Online Forum Leukaemia Care is involved in campaigning for patient well- Our online forum, being, NHS funding and drug www.healthunlocked.com/ and treatment availability. If you leukaemia-care, is a place would like an update on any of for people to ask questions the work we are currently doing or anonymously or to join in the want to know how to get involved, discussion with other people in a email advocacy@leukaemiacare. similar situation. org.uk Patient and carer conferences Patient magazine Our nationwide conferences Our magazine includes provide an opportunity to inspirational patient and carer ask questions and listen to stories as well as informative patient speakers and medical articles by medical professionals: professionals who can provide www.leukaemiacare.org.uk/ valuable information and support. communication-preferences/

Helpline freephone 08088 010 444 5 What is essential thrombocythaemia (ET)?

ET is a chronic condition Organisation who updated their characterised by too many classification in 2016 to include platelets in the blood. It belongs MPNs such as ET. to a group of conditions called Blood cells which are produced myeloproliferative neoplasms from stem cells in the bone (MPNs), which also includes marrow include: polycythaemia vera (PV) and myelofibrosis (MF). 1. Red blood cells (to carry oxygen to the tissues of your body) If you would like more 2. White blood cells (to fight information about PV and infection and disease) MF, you can download our booklets from our website 3. Platelets (to help blood to clot) at www.leukaemiacare. org.uk or request a copy of Production of new blood cells is the booklets by emailing very closely controlled in the bone support@leukaemiacare. marrow so that it is balanced org.uk or calling the with the loss of worn-out cells or helpline on 08088 010 cells lost by bleeding or damage. 444. In people with ET, there is an excess of platelets. Although it is platelets that are primarily There has been some debate affected, white blood cell levels about whether or not MPNs are may also be elevated. types of cancer. This is because the word neoplasm (which means In patients with ET, the blood new growth) is a term used for becomes thicker than normal and cancers (malignant neoplasms) the excess number of platelets and noncancerous tumours may cause blood clots to form (benign neoplasms). In ET there is more easily. Clots can block blood an uncontrolled increase in cells; flow through veins and arteries, therefore, many haematologists potentially leading to heart and cancer organisations do attacks or strokes. consider MPNs as blood cancers. ET is also associated with an This includes the World Health increased risk of bleeding

6 www.leukaemiacare.org.uk complications. your lifetime and is not present at birth. In some cases, ET can transform to MF or acute myeloid leukaemia In addition, a mutation in a gene (AML). known as CALR is present in 15% to 30% of patients with ET, and the Who is affected by ET? MPL mutation is seen in 4% to 8% of patients. ET is considered to be a rare disease. The number of people Some 10% to 20% of patients with diagnosed with ET in Europe is 0.6 ET do not express any of these per 100,000 patients per year. This three mutations. This is referred may explain why you might not to as triple-negative. have heard of ET or met anyone with the condition before. It is also important to be aware that, although some families ET is most common in those aged seem to develop the disease over 60 years. more readily than others, ET is usually not inherited nor passed It is more common in women on from parent to child. However, than men, with two women and this is less common, an being diagnosed for every man, increased likelihood of the gene particularly in patients who mutating (which can then lead to are less than 60 years of age. developing ET) can be inherited However, it must be noted that and is called familial ET. It 20% of patients are older than 40 resembles non-familial ET both in years of age at diagnosis. terms of clinical symptoms and What causes ET? mutations. While the exact cause of ET is Finally, some researchers believe not known, research has found MPNs may also be triggered that approximately 55% of people by past exposure to ionising who have ET have a mutation in radiation (a type of radiation a protein that regulates blood that has very high energy, like cell production. This protein is medical x-rays or nuclear fallout) known as Janus Kinase 2 (JAK2) or to some chemical substances and the mutation itself is JAK2 such as benzene and toluene. V617F. This mutation arises during

Helpline freephone 08088 010 444 7 Symptoms of ET

Many patients with ET do not • Reddish or purple skin have any symptoms when they Bleeding or clotting are diagnosed. Typically, these • patients are identified following • Unexplained bruising abnormal results during a routine full blood test for something else. This is not an exhaustive list If symptoms do develop, they tend of symptoms. There is a wide to do so over time. variety of symptoms that can be attributed to a diagnosis ET, so it If you have persistent symptoms is important to make your doctor of ET, make an appointment to aware of these. Not everyone will see your GP. Common symptoms have the same symptoms or to of ET include: the same degree of severity. It is also important to understand that • Fatigue the presence of these symptoms • Night sweats does not always indicate ET and they could be due to other Headaches (this can also • conditions and illnesses. include migraines with visual disturbances) In patients with ET, the increased numbers of platelets and white Itching • blood cells can result in blood • Fever clots (thrombosis) in a vein or artery, or they may cause bleeding • Swollen spleen (located under (haemorrhage). the ribs on the left of the abdomen, this organ helps to Blood clots or bleeding are a rid the body of toxins, waste and major complication of ET. There other unwanted materials) is a 1% to 3% risk of arterial or venous blood clots occurring in • Bone pain a patient per year; however, if the • Weight loss patient has a JAK2 mutation, this risk increases to 7.7%. • Dizziness or light headedness The blood clots prevent the

8 www.leukaemiacare.org.uk blood from flowing and the most you will then have the functioning common complications caused by of your platelets tested. Your these blood clots are: haematologist will closely monitor any antiplatelet drugs or Heart attacks, strokes or • anticoagulant drugs you may be damage to the gastro-intestinal receiving. Any significant bleeding tract due to blood clots in the episodes will be managed with arteries. tranexamic acid, which stops • Venous thrombosis such as bleeding in the short term, or a deep vein thrombosis in the transfusion of platelets. calf. General risk factors that can • Pulmonary embolisms, where a increase your likelihood of blood clot in the vein travels through clots or bleeding include: the blood stream and causes a • Being older than 60 years blockage in one of the arteries in the lungs. • Previous blood clots or bleeding The risks of bleeding are less • A high platelet count clearly defined, mostly because JAK2 mutations the definition of bleeding in • various studies is different. • Having a cardiovascular risk Risks of bleeding are known to factor, such as high blood be increased if the platelet count pressure, diabetes, smoking or is very high (between 1000x109/L high cholesterol and 1500x109/L). It could also be related to acquired von Willebrand Enlargement of the spleen is syndrome with extreme levels of present in 10% to 20% of patients platelets. with ET at diagnosis.

If you have experienced some bleeding, you may be screened for acquired von Willebrand syndrome. If the test is negative,

Helpline freephone 08088 010 444 9 How is ET diagnosed?

ET is often suspected if a routine tests including: blood test shows that a patient Blood tests – Blood tests can has a high platelet count, also • identify an increase in blood called thrombocytosis. cells and exclude other causes To make a diagnosis of ET, the of a high cell count. following criteria must be present: • Gene mutation analysis – • A platelet count of 450x109/L or This is to identify any gene higher mutations you may have, particularly JAK2, CALR or MPL. • A bone marrow biopsy showing an increase in the • Bone marrow investigations numbers of enlarged, mature – You may have either a bone megakaryocytes (cells in the marrow aspiration or both an bone marrow that produce aspiration and a bone marrow platelets) biopsy. This will be done to look for classic signs of ET. During Not meeting the diagnosis for • a bone marrow aspiration, the another MPN, such as PV or MF doctor or nurse takes some • The presence of a JAK2, CALR or bone marrow cells up into a MPL mutation syringe. A bone marrow biopsy is when they remove a one to two- If none of the mutations are centimetre core of bone marrow present, other causes of primary in one piece using a trephine or secondary thrombocytosis (a surgical instrument with a as well as diagnoses of PV and cylindrical blade). The sample is MF must be excluded. Primary then sent to the laboratory for refers to a condition that is the testing. root cause of the illness, whereas secondary refers to a condition that has developed as a result of another one.

ET is diagnosed using laboratory

10 www.leukaemiacare.org.uk What is the prognosis of ET?

Treatment is focussed mainly on or MPL does not affect the survival preventing any complications of patients with ET. However, older from blood clots or bleeding age, a high white blood cell count which is associated with ET. Low- and a previous history of blood risk patients are best managed clots and anaemia are associated with low-dose aspirin or watch with a poor survival. and wait alone, and patients with In some patients, ET can develop high-risk ET will benefit mainly into MF or AML. However, this is with cytoreductive therapy and rare. Transformation to AML risk anticoagulation. factors include: When properly monitored and Advanced age treated, patients with ET have • an excellent prognosis and life • Leucocytosis (a high white expectancy. Your prognosis can blood cell count) be influenced by a number of factors including your individual • Exposure to previous situation, your health history and treatments which suppress the way you respond to treatment. the bone marrow, such as ET is a chronic condition; chemotherapy therefore, it is important for you The prognosis of patients to have a positive partnership with ET can vary widely. Your between your haematology team. haematologist is the best person See your haematologist regularly to advise you based on your and report any new or different individual circumstances. symptoms.

For the majority of patients whose ET does not progress to MF or AML, a normal or very slightly reduced life expectancy can be anticipated.

Having the mutations JAK2, CALR

Helpline freephone 08088 010 444 11 Treating ET

Overview of treatment blood clots. Most treatments for ET are Medications intended to manage your symptoms and prevent any Cytoreductive therapy associated problems from blood Cytoreductive therapy is clots or bleeding in order to important in enabling ET and its maintain your quality of life. complications to be controlled. It Treatments for ET are aimed is the only treatment significantly at lowering the production of associated with reducing the platelets and maintaining normal occurrence of blood clots. In blood counts. This is called patients with ET, first-line cytoreductive therapy. There are a treatment is hydroxycarbamide, number of medications which can with second-line treatment being achieve this. anagrelide. Treatment according to Hydroxycarbamide (also known risk factors as hydroxyurea) This is the most commonly used Treatment will be based on an chemotherapy drug to treat ET assessment of your risk factors and is available as a tablet or regarding blood clots and capsule. bleeding. While cytoreductive therapy is fundamental in An early study of patients with treating ET, managing any high-risk ET confirmed that potential blood clots or bleeding the percentage of patients is equally important. Your receiving hydroxycarbamide had medical team will give you all the significantly less blood clots information about the treatment or bleeding (3.6%) compared which is best for you. with patients not receiving any treatment (24%). In recent Low-dose aspirin and controlled studies of patients anticoagulants may be used to with high-risk ET, neither prevent any blood clots, especially anagrelide nor interferon alpha if you have a history of developing could be shown to be superior to

12 www.leukaemiacare.org.uk hydroxycarbamide for reducing developing into AML. blood clots or bleeding. Anagrelide Hydroxycarbamide can cause Taken as a capsule, anagrelide is a mild side effects such as: drug that prevents the maturation • Increased risk of infection of platelets. It is used to counter the overproduction of platelets. It • Bruising or mild bleeding lowers the platelet count and has • Anaemia (as it reduces all types some effect on the red blood cells. of blood cells, including red Anagrelide is approved for blood cells) reducing elevated platelet counts • Fatigue in at-risk patients with ET who are intolerant to their current therapy • Diarrhoea or constipation or whose elevated platelet counts are not reduced to an acceptable Sore mouth • level by their current therapy. Changes to the skin, including • Side effects of anagrelide include: ulceration and increased risk of developing skin cancer • Headaches Hydroxycarbamide may also • Diarrhoea affect fertility. If you are taking it, you will be advised not to get • Palpitations pregnant or father a child, as • Fluid retention there may be a risk of harming the developing baby. It is advisable to Interferon alpha use effective barrier contraception Interferon alpha is a substance while taking the drug and also which occurs naturally in the body for a few months afterwards. If and reduces the rate at which hydroxycarbamide is used either blood cells, including platelets, alone or in combination with are made in the bone marrow. It other chemotherapy drugs over a can be made into a medicine to long period of time, it may slightly be given as an injection under increase the chance of the ET the skin to treat a wide range of

Helpline freephone 08088 010 444 13 Treating ET (cont.)

conditions, including ET. scarring. Side effects of interferon alpha New treatments include: • JAK2 inhibitors – JAK2 • Flu-like symptoms inhibitors block the function of the JAK2 mutation which slows Headaches • down blood cell production, • Vision disturbances reduces spleen size and improves symptoms. Up to 55% • Depression of patients with ET have been • Liver and thyroid disease found to have a JAK2 mutation. JAK2 inhibitors are being looked However, it does not increase the into as a treatment for ET. risk of secondary leukaemia and can be used in pregnancy. Prevention of blood Busulfan clots and bleeding Aspirin Busulfan is usually given to patients who have side effects As well as being used for reducing when taking hydroxycarbamide. pain and lowering temperature, It can be given as a tablet. Like aspirin is known to prevent hydroxycarbamide, busulfan platelets sticking together affects the bone marrow directly and may reduce your risk of and can lead to a fall in the blood developing a blood clot. cell counts. Its main side effects In patients with very low-risk are nausea and low platelet ET, aspirin might be required. counts. In patients with low-risk ET, Your haematologist can advise treatment with low-dose aspirin is you if busulfan is suitable, advised, especially if the patient and will monitor your progress has cardiovascular risk factors, as carefully during your treatment. it has been shown to reduce both There is evidence that busulfan venous and arterial blood clots. can increase the risk of secondary Research shows that aspirin is leukaemia and can cause lung very effective at reducing risks of

14 www.leukaemiacare.org.uk heart attacks and strokes in many a venous blood clot that has people with different levels of risk. occurred with no identifiable risk Patients with intermediate-risk ET factor. This will be done after your and high-risk ET will be given low- bleeding risk has been assessed. dose aspirin whether they have Patients with ET and other MPNs cardiovascular risk factors or not. more commonly have unusual Low-dose aspirin can have some sites of venous clots, meaning side effects such as bleeding and that the use of anticoagulation indigestion. It can also cause treatment can be particularly gastric irritation, bleeding in the beneficial for them. These unusual stomach, and ulcers. You may sites of venous clots include: find that you bruise more easily The splanchnic vein – this and that you bleed for a long • drains the blood from the time if you cut yourself. Applying stomach, pancreas, spleen and pressure to any small cut or intestines. wound with a sterile bandage will stop the bleeding. Aspirin can • The cerebral vein – this drains make the symptoms of asthma blood from the brain. worse for those who suffer with it. In addition, some people can be allergic to aspirin.

Your haematologist will tell you if aspirin is safe for your particular situation and may suggest other similar medications, such as dipyridamole or clopidogrel, if necessary. Anticoagulation treatment Long-term anticoagulation treatment uses medication, also referred to as blood-thinners, to help prevent blood clots. This can be started if you have had

Helpline freephone 08088 010 444 15 Living with ET

After a diagnosis of ET, you may make changes to your lifestyle find that it affects you both to try to stay as well as possible physically and emotionally. This after your diagnosis and during section will talk about both of treatment. Don’t try to change too these aspects. much at once. Adopting a healthy way of living is about making Emotional impact of ET small, manageable changes to Being told you have cancer can your lifestyle. be very upsetting. Although the A healthy lifestyle includes having outlook for many ET patients is a a well-balanced diet and being positive one, it is a blood cancer physically active. With some and a rare condition and, because of the side effects you may be of this, you may need emotional, experiencing, the idea of getting as well as practical support. Being out and being active may be the diagnosed with a rare disease can last thing you want to do, but it affect the whole of you, not just is important to try and stay as your body, and can impact you active as possible to make you emotionally at any point of your feel better and reduce some of the journey. It is likely that you will symptoms or side effects you may experience a range of complex be experiencing. thoughts and emotions, some of which may feel strange or unfamiliar to you. It is important "People with an MPN to know that these feelings are all should not smoke valid and a normal response to and should exercise your illness. to maintain their general fitness and It is important to remember that, improve their vitality with current treatments, you can and cardiovascular expect a good response and to live performance – this a long, normal life. also reduces their risk of cardiovascular Looking after you disease." – Professor You can live a long and normal Claire Harrison life with ET, but you may want to

16 www.leukaemiacare.org.uk One of the most commonly • Discuss your fatigue with your reported side effects from the doctor or nurse. treatment of ET is fatigue. This isn’t normal tiredness and doesn’t Practical support improve with sleep. Work and finances Some general tips on how to deal Being diagnosed with ET can with fatigue include: sometimes lead to difficulties relating to your work life. Your Have a regular lifestyle – try • diagnosis may lead to temporary going to bed and waking up sick leave or a reduction in approximately the same time working hours but it can also every day and try to avoid lying mean that you have to stop work in. altogether. You may need to • Take part in regular, gentle make an arrangement with your exercise to maintain your employer for times when you may fitness levels as much as need to go into hospital or for possible. those times when you may not be well enough to go into work. • Reserve your energy for what you find important and build Your consultant or your GP can rest periods around those times. arrange letters for your employer to confirm your diagnosis and the • Before going to bed, avoid effects it may have on your work stimulants such as alcohol, life. It is often worth taking time to coffee, tea or chocolate, or explain ET to your employer, as it using laptops, tablets or mobile is likely they will never have heard phones. of the disease before.

• Keep your bedroom quiet and at It is important for you to know a comfortable temperature. that people with any form of • Talk about your worries with cancer are covered by law by family, friends, or your doctor the Equality Act. This means or nurse, or patient support that legally your employer groups. cannot discriminate against you and must make reasonable

Helpline freephone 08088 010 444 17 Living with ET (cont.)

arrangements and adjustments relating to your disease.

If you would like advice about some of the financial help available to you, then you can speak to our Patient Advocacy team on 08088 010 444. Alternatively, Macmillan has published a booklet about financial support following a diagnosis of cancer that might be useful to you. They can also give you personal advice over the phone via their helpline at 0808 808 000 and you can discuss which benefits you are eligible for. Some Macmillan centres can arrange face-to-face meetings with a benefits advisor. They can also provide financial assistance in the form of grants – ask your nurse in the hospital how to apply.

As ET is regarded as a cancer, you will also be entitled to apply for a medical exemption certificate which means that you are entitled to free NHS prescriptions in England and Wales. Your GP or specialist nurse at the hospital can provide you with the details of how to apply for this.

18 www.leukaemiacare.org.uk Helpline freephone 08088 010 444 19 Talking about ET

Talking to your • Be open and honest when you discuss your symptoms haematologist and how you are coping. Good ET is a rare condition. It is patient-doctor communication important for you to develop a tends to improve outcomes for good working relationship with patients. your haematologist so you are given the best treatment possible • Bring someone along to your for you. appointment. They can provide support, ask questions and take Here are some tips for working notes. well with your haematologist: • Don’t be afraid to ask for • If it is an initial consultation, a second opinion – most take along a list of your current haematologists are happy for medications and doses, and you to ask. a list of any allergies you may have. You need to tell your haematologist if... • If you have a complicated medical history, take a list of You’re having any medical diagnoses, previous procedures treatment or taking any products and/or complications. such as prescribed medicines, over the counter treatments • Make a list of questions to take or vitamins. It is important to to your appointment. This will understand that treatments, help the discussion with your including complementary haematologist. therapies, which are perfectly • It can be useful to repeat back safe for most people, may what you have heard so that you not be safe if you are being can be sure that you have fully treated for ET. Remember, if understood. you want to start any form of complementary therapy outside • Note down information to help of your medical treatment, you remember what was said. consult your haematology consultant or clinical nurse

20 www.leukaemiacare.org.uk specialist prior to beginning it. terms of what others say and do. It is important to understand Often people make assumptions the difference between and do what they think helps. For complementary therapies, used example, saying you look well, alongside standard treatment, recounting stories of others they and alternative therapies, used know with a similar diagnosis or instead of standard treatment. encouraging you to look ahead There is no evidence to suggest and stay positive, isn’t always that any form of alternative what people really want to hear. therapy can treat ET. In many ways, the more you Talking to other people communicate with them the better. However, it is perfectly Telling people you have a rare natural if you feel at some points condition like ET can be hard to that you would like to keep some explain. You might find it useful to things to yourself. let your close family and friends, as well as your employer, know These points may help you: about your health condition. It Explain that you have a might be easier to provide people • condition that means your with basic information and give bone marrow does not function them information leaflets about properly, and this affects ET if they want to know more in- the number of blood cells it depth details. produces. It is probably best to focus Explain your symptoms (maybe conversations on the symptoms • you are tired, or have a lot of that you are experiencing, how pain). the condition affects you and how you feel about it. Often • Explain what you need (maybe people misunderstand and, more help day-to-day, or unfortunately, it will mostly fall someone to talk to). to you to educate them as best as you can. Where possible, it’s advisable to let people know what you find helpful and unhelpful, in

Helpline freephone 08088 010 444 21 Talking about ET (cont.)

You could also consider the who care will want to help you following when telling people as best as they can. Talk as and about your diagnosis: when you feel comfortable, so those around you will know Find out more – Try to find • when you need them most. out as much as you can about your condition from If you’re struggling to reliable internet sources, come to terms with your charitable organisations or your diagnosis and prognosis, consultant haematologist. The you can speak to us on more you know, the more you our helpline. Call us on can share. 08088 010 444. • Have a print out to hand – It may help to have a factsheet, or a booklet like this one, to hand to share with family and friends. This will take the pressure off you having to remember everything they may want to know. • Explain your needs – Try and be clear about what your needs may be. Perhaps you need help with the weekly food shop, help with cooking dinner, or someone to drive you to and from appointments. You may find that friends and family are pleased that they can do something to help you. • Be open about how you feel – Don’t be afraid of opening up about how you feel, as people

22 www.leukaemiacare.org.uk Glossary

Acute Myeloid Leukaemia processes including regulation of (AML) calcium, cell adhesion and gene expression. Mutations in CALR are A rapid and aggressive cancer common in ET and MF. of the myeloid cells in the bone marrow. Chromosomes Anaemia Thread-like structures which carry the genes, and are located in the A condition where the number of nuclei of every cell in the body. red blood cells are reduced. Red There are 46 chromosomes (23 blood cells contain haemoglobin pairs) in humans. and transport oxygen to body cells. This may be due to a lack Chronic Myeloid Leukaemia of iron, leukaemia, or sickle cell (CML) disease. A leukaemia in which the myeloid Anticoagulation cells start multiplying in the bone marrow leading to large numbers The process of administering of abnormal, immature myeloid anticoagulants which are drugs cells called blasts, which prevent to prevent the blood from clotting the bone marrow from producing unnecessarily. enough healthy blood cells of all Bone Marrow types. A soft blood-forming tissue that First-line Treatment fills the cavities of bones and The first treatment given for contains fat, immature and a disease. It is generally the mature blood cells, including treatment accepted by the white blood cells, red blood cells medical establishment for initial and platelets. treatment of a given type and Calreticulin (CALR) stage of cancer. A soluble protein which is known to be involved in many body cell

Helpline freephone 08088 010 444 23 Glossary (cont.)

Genes Myelofibrosis (MF) Genes are made up of DNA which A reactive and reversible stores the genetic information process which occurs with many required to make human proteins. cancerous and non-cancerous diseases of the bone marrow. Irradiation Particles or rays falling on to a Platelets surface (radiation wave on the One of the types of blood cells surface of the skin). which help to stop bleeding. Leukaemia Polycythaemia Vera (PV) A group of cancers that usually A chronic condition belonging begin in the bone marrow to the myeloproliferative and result in high numbers of neoplasms group of diseases. It abnormal blood cells. These cells is characterised by too many red are not fully developed and are blood cells, and sometimes too called blasts or leukaemia cells. many platelets and white cells, in Depending on the type of blood the blood. cell involved, there are different types of leukaemia with varying Prognosis characteristics, such as being An indication of how well a acute (develops quickly) or patient is expected to respond chronic (develops slowly). to treatment based on their individual characteristics at Myeloproliferative Neoplasms the time of diagnosis or other (MPNs) timepoint in the disease. A disease of the bone marrow in which excess cells are produced. Pulmonary Embolism A blockage of a blood vessel in the Myeloid lung. It can be secondary to a clot Relates to bone marrow. elsewhere in the body which then travels up to the lung.

24 www.leukaemiacare.org.uk Red Blood Cells excessive number of platelets in the blood (a platelet count greater Small blood cells that contain than 450x109/L). Platelets are haemoglobin and carry oxygen blood cells in plasma that stop and other substances to all bleeding by sticking together to tissues of the body. form a clot. Too many platelets Second-line Treatment can lead to certain conditions, including stroke, heart attack, or a Treatment other than the type clot in the blood vessels. used the first time (first-line treatment). Thrombosis Spleen Clotting or coagulation of the blood in a part of the circulation The largest organ of the lymphatic in both arteries and veins. system whose function is to help rid the body of toxins, waste and von Willebrand syndrome other unwanted materials. The A common hereditary blood- spleen is located under the ribs clotting disorder which causes on the left of the abdomen. varying degrees of bleeding. It Stem Cell can be hereditary or acquired. In acquired von Willebrand The most basic cell in the body syndrome, the patient has auto that has the ability to develop antibodies to the von Willebrand into any of the body’s specialised clotting factor and this often cell types, from muscle cells to occurs in conjunction with brain cells. However, what makes another autoimmune disease these stem cells reproduce such as rheumatoid arthritis. uncontrollably, as in cancer, is thought to be linked to White blood cells chromosome abnormalities. White blood cells are one of the Thrombocytosis types of cells found in the blood and bone marrow, along with red A condition in which there is an

Helpline freephone 08088 010 444 25 Glossary (cont.)

blood cells and platelets. White blood cells create an immune response against both infectious disease and foreign invaders. Granulocyte white blood cells, include the neutrophils (protect against bacterial infections and inflammation), eosinophils (protect against parasites and allergens) and basophils (create the inflammatory reactions during an immune response). Other white blood cells include the lymphocytes (recognise bacteria, viruses and toxins, to which they produce antibodies) and monocytes (clear infection products from the body).

26 www.leukaemiacare.org.uk Useful contacts and further support

There are a number of helpful Blood Cancer UK sources to support you during Blood Cancer UK is the leading your diagnosis, treatment and charity into the research of blood beyond, including: cancers. They offer support to • Your haematologist and patients, their family and friends healthcare team through patient services. • Your family and friends 0808 2080 888 • Your psychologist (ask your www.bloodcancer.org.uk haematologist or CNS for a Cancer Research UK referral) Cancer Research UK is a leading Reliable online sources, • charity dedicated to cancer such as Leukaemia Care research. Charitable organisations • 0808 800 4040 There are a number of www.cancerresearchuk.org organisations, including ourselves, who provide expert Macmillan advice and information. Macmillan provides free practical, Leukaemia Care medical and financial support for people facing cancer. We are a charity dedicated to 0808 808 0000 supporting anyone affected by www.macmillan.org.uk the diagnosis of any blood cancer. We provide emotional support Maggie’s Centres through a range of support Maggie’s offers free practical, services including a helpline, emotional and social support patient and carer conferences, to people with cancer and their support group, informative families and friends. website, one-to-one buddy service and high-quality patient 0300 123 1801 information. We also have a nurse www.maggiescentres.org on our help line for any medical Citizens Advice Bureau (CAB) queries relating to your diagnosis. Offers advice on benefits and Helpline: 08088 010 444 financial assistance. www.leukaemiacare.org.uk [email protected] 08444 111 444 www.adviceguide.org.uk

Helpline freephone 08088 010 444 27 Leukaemia Care is a national charity dedicated to providing information, advice and support to anyone affected by a blood cancer.

Around 34,000 new cases of blood cancer are diagnosed in the UK each year. We are here to support you, whether you’re a patient, carer or family member.

Want to talk?

Helpline: 08088 010 444 (free from landlines and all major mobile networks) Office Line: 01905 755977 www.leukaemiacare.org.uk [email protected]

Leukaemia Care, One Birch Court, Blackpole East, Worcester, WR3 8SG

Leukaemia Care is registered as a charity in England and Wales (no.1183890) and Scotland (no. SCO49802). Company number: 11911752 (England and Wales). Registered office address: One Birch Court, Blackpole East, Worcester, WR3 8SG