Acute Promyelocytic Leukaemia (APL)

A Guide for Patients Introduction

Being diagnosed with acute promyelocytic leukaemia (APL) can be a shock, particularly when you may never have heard of it. If you have questions about APL – 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 about how to get the best from your haematologist, plus practical advice on how to help important people in your life understand such a rare condition.

For more personalised peer reviewed by Nigel Russell, information, talk to your Professor of Haematology at haematologist, clinical nurse University of Nottingham, and specialist or hospital pharmacist. James Allan, Professor of Cancer Genetics at Newcastle University. This booklet was originally We are also grateful to Thea compiled by Ken Campbell, MSC Wilson for her contributions as a (Clinical Oncology) and peer patient reviewer. reviewed by Dr George Cherian and Professor David Grimwade, Throughout this booklet, you will who provided additional support. see a number of quotations. These The rewrite was put together by are the real experiences of Lisa Lovelidge and updated by cancer patients and may help you our Patient Information Writer, to understand your disease and Isabelle Leach. It has been situation a bit better.

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

Version 2 Printed: 01/2020 2 www.leukaemiacare.org.uk Review date: 01/2022 In this booklet

Introduction 2

In this booklet 3

About Leukaemia Care 4

What is APL? 6

Symptoms and diagnosis of APL 8

Treating with APL 10

Living with APL 18

Talking about APL 22

Glossary 25

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 APL?

Acute promyelocytic leukaemia to all tissues of the body. (APL) is a rare sub-type of acute that form blood clots myeloid leukaemia (AML) in which 2. to stop bleeding. there is an increased production of immature, abnormal white 3. White blood cells that fight blood cells called promyelocytes infection and disease; the in the . The term shortest-lived white cells are acute indicates that the called . leukaemia develops rapidly rather than being a description of its A lymphoid stem cell becomes a seriousness. lymphoblast cell and then one of three types of lymphocytes (white To understand APL, it is helpful blood cells): to understand how blood cells are normally produced. Blood 1. B-lymphocytes that make cells are produced in the bone antibodies to help fight marrow, which is spongy tissue infection. found inside bones. Each day, 2. T-lymphocytes that help the bone marrow produces more B-lymphocytes make the than a trillion new blood cells to antibodies that help fight replace those that are worn out. infection. Blood stem cells divide to produce either mature blood cells or more 3. Natural killer cells that attack stem cells. Only about one in 5000 cancer cells and viruses. of the cells in the bone marrow is In APL, the myeloid cells that are a stem cell. A blood stem cell, also destined to mature into white called a haematopoietic stem cell, blood cells (through a process may become a myeloid stem cell called differentiation) fail to or a lymphoid stem cell. do so and remain as immature A myeloid stem cell becomes one promyelocyte cells. of three types of mature blood These promyelocyte cells cell: accumulate in the bone marrow 1. Red blood cells that carry leading to a shortage of normal oxygen and other substances white and red blood cells and

6 www.leukaemiacare.org.uk platelets which cause the gene on chromosome 17 swap and symptoms of APL. fuse to become the PML-RARA gene. How common is APL? A small number of APL patients APL is considered a rare disease (less than 10%) do not have the as it affects about two people PML-RARA gene but have other in every million per year. It rarer gene fusions. APL can also represents approximately 10% of develop after receiving certain all cases of AML. APL affects men types of chemotherapy or and women equally. There are radiotherapy given for another about 160 new cases diagnosed in cancer. This type of APL is called the UK each year. treatment-related APL. The risk of APL can be diagnosed at any age. developing APL is highest around The median age at diagnosis is 51 three years after the treatment for years. the prior cancer. What causes APL? The exact cause of APL is not known. However, APL is commonly associated with the PML-RARA gene which is sometimes called the hallmark of APL. The PML- RARA gene is created during a person’s lifetime and is not passed on to children. It is the result of a translocation between chromosome 15 and chromosome 17. A translocation is the transfer of one part of a chromosome to another part of the same or a different chromosome, resulting in rearrangement of the genes. In the case of APL, the PML gene on chromosome 15 and the RARA

Helpline freephone 08088 010 444 7 Symptoms and diagnosis of APL

Symptoms of APL DIC may happen in other forms of leukaemia but is rare. It affects The most common symptoms about four out of five patients of APL are similar to those seen with APL. DIC happens when the in other acute leukaemias and body forms clots and breaks down are caused by the bone marrow clots inside the blood vessels. This failing to produce enough normal uses up the clotting factors in the blood cells. Most patients will blood and this can lead to severe, experience shortness of breath even life-threatening, bleeding. and tiredness with slight exercise, Fortunately, modern treatments which is caused by a shortage are very effective in blocking DIC. of red blood cells (anaemia). Anaemia means the body cannot To summarise, common supply enough oxygen to muscles symptoms and their causes are: and other tissues. Infections are Anaemia – breathlessness, common and often persistent • easily tired because of a deficiency in neutrophils, a • Low white blood cells – that normally helps to fight frequent and persistent infection. infections Bruising and bleeding are often • Low platelets – bruising and/or seen, and can range from slight bleeding bruises in the skin to serious internal bleeding. This is caused • DIC – bruising/bleeding which partly by a shortage of platelets, may be very severe but also by a condition called disseminated intravascular Diagnosis of APL coagulation (DIC). Platelets help If APL is suspected, you’ll have the body to form a clot at the site a set of tests to confirm the of bleeding, so when there are too diagnosis. If you’re diagnosed few, this may cause bruising or with APL, you will have further slight bleeding. tests to determine the right treatment for your cancer. It’s important that you know and

8 www.leukaemiacare.org.uk understand your diagnosis, so you and RARA are two genes which can ask questions and be fully are normally found on different informed of what to expect. chromosomes. In APL, the two chromosomes swap over part of Full blood count their DNA, which joins the PML A blood sample is examined and RARA genes together. This using an automatic cell-counting test is important because the machine and by examining a main drugs used to treat APL work stained film under a microscope. directly on the PML-RARA gene. In The cell-counter will usually the very rare cases of APL without indicate that there are large the gene, other treatments can be numbers of abnormal white cells used. in the blood. The appearance of There are also a number of other the stained blood cells is usually special investigations that can very typical of APL. be done to confirm the diagnosis Bone marrow examination of APL and to help in planning If the results of a blood sample treatment. Unlike most forms of show that you may have APL, or leukaemia, treatment of APL often another form of leukaemia, a bone starts before all the tests are marrow sample will be taken, completed. usually from the hip bone. This is These tests may be repeated done under a local anaesthetic from time to time during your and does not take very long. The treatment. This is to find out bone marrow sample is important how the APL is responding to to confirm the diagnosis and also treatment. for comparison with later samples to show how APL is responding to "Lots of things were thrown at me treatment. in a short amount of time but the sentence I clung on to was ‘we Additional investigations are waiting confirmation, but the There is a test that looks for an leukaemia we think you have carries abnormality called PML-RARA. This a high cure rate. We should know by is an abnormal fusion gene – PML the morning.’ I remember thinking, please, please let it be that one."

Helpline freephone 08088 010 444 9 Treating APL

APL is treated in a very different symptoms. A firm diagnosis of way from other forms of AML; APL using genetic testing can be if a patient with APL is given performed later, and treatment standard treatment, there is a can be discontinued if APL is not risk of serious problems with confirmed. their clotting system. Fortunately, Patients with APL are generally it is usually very easy to tell the subdivided into the following difference between APL and other two groups according to their types of AML. white blood count as treatment APL usually responds very well to recommendations can differ for treatment and patients with this each group: form of leukaemia have a good Low- to intermediate-risk: chance of being cured. 1. patients with a white blood APL can affect people of any cell count of 10,000 cells per age, but this booklet is about microlitre of blood or less. APL in adults. If you are a parent High-risk: patients with a of a child with APL, you should 2. white blood cell count of ask their specialist about the more than 10,000 cells per differences in treatment and microlitre of blood. outlook for children.

If you have any concerns, contact First-line treatment your haematologist. First-line treatment for APL includes all-trans retinoic acid Treatment options (ATRA), which is an active by- Treatment of patients who are product of vitamin A. ATRA blocks suspected of having APL should the effect of the PML-RARA gene be treated immediately, even that prevents the promyelocyte before the diagnosis is made, cells maturing into normal white because they can quickly develop blood cells (differentiation). ATRA potentially life-threatening is not a chemotherapeutic drug bleeding or blood clotting and is called a differentiating

10 www.leukaemiacare.org.uk agent. It is given in combination it to be at least as effective as with another drug in patients the combination of ATRA and with APL to prevent any drug anthracyclines, if not more so, resistance. with a reduced risk of disease relapse. ATRA can sometimes be given with chemotherapy drugs called Induction treatment anthracyclines. Anthracyclines, To achieve remission (induction such as daunorubicin and therapy), the 2019 guidelines idarubicin, interfere with the DNA from the European Leukaemia and reproduction of white blood Network (European LeukemiaNet) cells, including the leukaemia recommend the following cells. ATRA is given as a capsule, regimens: while anthracyclines are given intravenously. Low-to-intermediate risk patients: ATRA and ATO In 2018, NICE approved a drug called arsenic trioxide (ATO) for High-risk patients: Both of the the first-line treatment of APL in following regimens achieve previously untreated patients, similar results; however, ATO with low- to intermediate-risk is not approved for high-risk disease and patients whose APL patients by NICE as yet. has returned (relapsed) or did ATRA and ATO plus a not respond to chemotherapy • cytoreductive chemotherapy (refractory). ATO is also a such as cytarabine. differentiating agent and acts in a Cytoreductive means that the similar way to ATRA. chemotherapy reduces the Because differentiating agents number of cells. have less side effects to ATRA plus anthracyclines. The chemotherapy drugs, especially • most frequently used regimen anthracyclines, the combination being called AIDA. of ATRA and ATO alone is a preferred first-line therapy, The treatment for APL that has particularly as studies found developed as a consequence of

Helpline freephone 08088 010 444 11 Treating APL (cont.)

prior chemotherapy is normally to three courses of anthracycline- similar to APL associated with based chemotherapy should be the PML-RARA gene, although given for consolidation therapy. your doctor may choose to use a This is usually given as an different drug in this situation. inpatient. In addition to induction Minimal residual disease and treatment, patients with APL molecular monitoring require supportive care in After consolidation treatment the form of blood product has been completed, a bone transfusions to maintain the marrow molecular assessment count and the blood is recommended to confirm clotting indicators as normal complete response (CR) with as possible and to prevent the negative minimal residual risk of bleeding. Blood chemical disease (MRD): levels (particularly potassium and magnesium which are important • Complete response (CR), for electrical conduction in the also known as complete heart) will be monitored closely. remission – This is based Sometimes it is necessary to on morphological remission, also give potassium and/or meaning that a patient has a magnesium supplements. normal blood count, a normal development of blood cells in Consolidation treatment the bone marrow and their blast To consolidate remission count is at less than 5%. in patients who have not received chemotherapy-based • Minimal residual disease treatment, four courses of ATO (MRD) - The presence of small, and seven courses of ATRA are but measurable, numbers of recommended. This can usually leukaemia cells in the blood be given as an outpatient. of a patient who does not have any clinical presentation of a For patients who received ATRA blood cancer (i.e. the patient and chemotherapy regimens, two is in remission). If there are

12 www.leukaemiacare.org.uk no detectable leukaemia cells successful in most patients with found, then a patient is said to APL. However, for patients who be MRD-negative. haven’t gone into first remission or who have relapsed, second-line This is then to be followed by treatment options are available. routine monitoring for certain patients, depending on their CR Relapse or being refractory to and MRD results as well as their first-line treatment can occur in risk classification: any patient with APL, regardless of whether they have been treated Routine monitoring may not be • with ATRA with ATO or ATRA with necessary for low-risk patients chemotherapy. However, these with CR with negative MRD after events are uncommon in low- to consolidation. intermediate-risk patients. MRD monitoring every three • The second-line treatment you months for up to three have for relapsed or refractory APL years after consolidation is will depend mainly on which first- recommended for high-risk line treatment you were given. If patients. you have had ATRA with ATO as Maintenance treatment first-line treatment, then you will receive ATRA with chemotherapy, For low- to intermediate-risk and vice-versa (you will be given patients, maintenance treatment ATRA with ATO if you had ATRA after consolidation with ATO and with chemotherapy as first-line ATRA is not recommended, but for treatment). high-risk patients on ATRA and chemotherapy who are showing In young, fit patients, an clinical benefit, maintenance may autologous stem cell transplant be initiated with tablets for two can be performed. With an years. autologous stem cell transplant, you are given intensive Second-line treatment chemotherapy to destroy all the First-line treatment is generally leukaemia cells. However, as the

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

chemotherapy will also kill your blood cells, red blood cells and own bone marrow cells, you are platelets) given a transplant of your own healthy stem cells which were Differentiation syndrome collected before the intensive Previously known as retinoic chemotherapy. However, in acid syndrome (or RA syndrome), patients who were responding differentiation syndrome can well to ATO and then relapsed, affect some patients being a transplant is not always treated with ATRA, ATO or both. It necessary. occurs when these differentiating agents start allowing the Side effects promyelocyte cells to mature. Side effects in patients with The exact mechanism involved is APL when treated with ATRA unknown but it is thought to be and ATO include differentiation an inflammatory response. syndrome, pseudotumour cerebri, Differentiation syndrome is most hyperleukocytosis, heart rhythm likely to happen during the first disturbance (prolongation of QTc three weeks of treatment and is interval) and signs of toxicity in more common in patients with a the liver. high white blood cell count.

Side effects common Symptoms include: with anthracyclines are cardiomyopathy (disease of the • Fever heart muscle) and secondary Cough leukaemia. • Breathing difficulty Other side effects include: • Fluid in the lungs • Nausea and vomiting • Weight gain • Alopecia • Fluid in the tissues • Bone marrow suppression • (resulting in low levels of white • Kidney damage

14 www.leukaemiacare.org.uk Differentiation syndrome can Hyperleukocytosis, usually be treated with steroids. leukostasis and disseminated Treatment with differentiating intravascular coagulation agents may be temporarily Hyperleukocytosis is said to reduced or suspended, but occur when the white blood cell this would only happen if the count is greater than 100,000 per differentiation syndrome is very microlitre. This highly increased severe, which is rarely the case. white blood cell count often Most current ATRA with ATO leads to complications such as regimens include preventative leukostasis and disseminated steroid treatment. intravascular coagulation. Pseudotumour cerebri Leukostasis occurs when the Pseudotumour cerebri is seen excessive numbers of white blood with both ATRA and ATO, and cells make the blood viscous and is more common when these prone to clotting. two differentiating agents are given together. Pseudotumour Disseminated intravascular cerebri is a non-harmful increase coagulation (DIC) is a condition in pressure in the skull and where the small blood clots unrelated to any tumour. develop throughout the bloodstream and block blood Symptoms include: vessels. Because the increased clotting uses up all the platelet Headaches • cells and clotting factors to • Swelling of the optic disk control bleeding, patients are prone to excessive bleeding. This • Double vision is a very serious complication • Confusion which should be treated as soon as possible. If it occurs, pseudotumour cerebri can be treated with steroids and In order to prevent DIC from by decreasing the dose of ATRA. developing, patients with hyperleukocytosis should be

Helpline freephone 08088 010 444 15 Treating APL (cont.)

treated with anthracycline- What happens if these based chemotherapy. The use of the chemotherapy drug drugs do not work for hydroxycarbamide should be me? considered for treatment of For the majority of patients leukocytosis (a white blood cell who do not respond to first-line count greater than 100,000 per treatment with ATRA and ATO, or microlitre) in patients treated ATRA with ATO and cytarabine in with combination therapy of ATRA high-risk patients, or patients and ATO to keep the white blood who relapse at a later point, a cell count low. good response to second-line Heart rhythm disturbances treatment is generally achieved with ATRA and anthracyclines, Some patients treated with ATRA, or ATRA with ATO if they received ATO or both might show heart ATRA with anthracyclines as rhythm disturbances such as a first-line treatment. While prolongation of the QTc interval relapse or being refractory to on their electrocardiogram first-line treatment can occur (ECG) which may cause heart in any patient with APL, this is problems, especially for patients an uncommon event in low- to with existing heart disease. intermediate-risk patients. Therefore, ECG monitoring before and twice a week throughout In the event of requiring second- treatment is recommended. For line treatment, it is worth patients who show prolongation considering an autologous stem of the QTc interval on their ECG, cell transplant in patients who treatment should be stopped and are fit enough to withstand the restarted when the heart rhythm procedure. disturbances subside. Your consultant will be able to discuss these treatments with you, and any circumstances in which they might become necessary.

16 www.leukaemiacare.org.uk Prognosis of APL For patients who are diagnosed and start treatment promptly, the outcome of APL is very good. Since the introduction of the combination of ATRA and ATO, cure rates for APL of approximately 90% for low- to intermediate-risk and 85% for high-risk patients have been reported.

An important remaining issue is early death which occurs in approximately 10-15% of patients who do not receive immediate treatment. Efforts in this area are focusing on education to improve prompt diagnosis and early management of patients with suspected APL.

Patients who are older, male, have problems with their kidneys (measured by serum creatinine levels) and increased levels of fibrinogen (protein involved in clotting the blood) have a worse prognosis.

Helpline freephone 08088 010 444 17 Living with APL

Being diagnosed with an aggressive form of leukaemia like APL can be difficult, both practically and emotionally. This chapter will talk about both of these aspects.

Emotional impact of know your limits and not try to change too much at once. Exactly APL what you can do will vary and Being told you have cancer can be will depend on the treatment you very upsetting. It can be especially have had, and how fit you were difficult with acute leukaemia before your leukaemia. Adopting as you often get ill suddenly and a healthy way of living is about have to start treatment quickly. making small, manageable There is usually very little time to changes to your lifestyle. take in information and start to cope with it. You can find more information about living well with APL is a rare condition and, leukaemia on our website: www. because of this, you may need leukaemiacare.org.uk/support- emotional, as well as practical, and-information/information- support. Being diagnosed with about-blood-cancer/living-well- a rare disease can impact you with-leukaemia/ emotionally at any point of your journey. It is likely that you will Diet experience a range of complex Diet plays an important part thoughts and emotions, some in coping with cancer and its of which may feel strange or treatment and recovery. A well- unfamiliar to you. It is important balanced diet can help you feel to know that these feelings are all stronger, have more energy, and valid and a normal response to recover quicker. your illness. If you’re having treatment, Looking after you you may notice that you lose weight, or your taste or appetite Following a diagnosis of APL, you changes. This may be due to the may wish to make changes to side effects of your treatment your lifestyle. It’s important to

18 www.leukaemiacare.org.uk including a sore mouth or nausea ordinary infections may occur and sickness. more often and be more severe or longer lasting. You may also get ill Once your treatment has finished from infections with germs that though, you should begin to feel normally live in your body without better and be able to eat a normal causing problems, but which grow diet. This can take a while after more rapidly when your immune intensive treatment. system is not working – these are Exercise called opportunistic infections. With some of the side effects The neutropenic diet can help you may be experiencing, such protect patients with weakened as fatigue, the idea of getting immune systems. out and being active may be the If you think you may have an last thing you want to do, but it infection, you should contact your is important to try and stay as doctor straightaway. Common active as possible. This will help to symptoms of infection include: make you feel better and reduce some of the symptoms or side • Fever – a raised temperature effects you may be experiencing. (38°C or higher) Speak to your clinical nurse specialist about exercises that • Aching muscles may be suitable for you. • Diarrhoea Infection • Headaches One of the most common Excessive tiredness problems following a diagnosis • of APL is infection. When you have The signs and symptoms of APL, your body is not able to fight infection may be less obvious infections as well as normal – this when you have APL, so if you are is known as immunosuppression. in any doubt it is best to contact If you have immunosuppression, your doctor and ask for advice.

Helpline freephone 08088 010 444 19 Living with APL (cont.)

You can help to reduce the risk of is recommended by someone infection by taking some simple other than your APL specialist, you precautions: should check that it is safe first. • Wash your hands frequently, Shingles especially after using the If you have previously been toilet, and also if you have exposed to chickenpox, you may touched something like a door develop a painful nerve condition knob or banister which can called shingles. Even if it was be contaminated with lots of a long time ago, the virus can germs. live dormant for many years • Try not to spend unnecessary and surface when your immune time in crowds; especially if system is supressed. You may there is an epidemic of flu or be able to receive a vaccine another illness. against shingles but, as it is a live vaccine, you should talk to your • You should be very careful to doctor about this. follow food safety advice, such as cleanliness in the kitchen Practical support and not keeping food after use- Work and finances by dates. Being diagnosed with APL means Vaccines you will need to start treatment Vaccinations may not work as well straightaway and so you, or when you have leukaemia, but it is someone you know, will need to still recommended that you have contact your employer to inform your annual flu vaccine. This will them of your situation. Your still reduce the risk of getting ill condition will mean that you will and will offer you some protection. need to be at hospital frequently at first and you will need to make APL patients should avoid having the appropriate arrangements live vaccines which are used for with your employer with regard to measles, mumps and rubella your working arrangements. (MMR) and shingles. If a vaccine

20 www.leukaemiacare.org.uk You may need to negotiate a help available to you, then reduction in working hours or you can speak to our Patient make an arrangement with your Advocacy team on 08088 010 employer for times when you have 444. Alternatively, Macmillan to go into hospital or for those has published a booklet about times when you may not be well financial support following a enough to go into work. diagnosis of cancer that might be useful to you. They can also Your consultant or your GP can give you personal advice over the arrange letters for your employer phone via their helpline at 0808 to confirm your diagnosis and the 808 0000 and you can discuss effects it may have on your work which benefits you are eligible life. It is often worth taking time to for. Some Macmillan centres can explain APL to your employer, as it arrange face-to-face meetings is likely they will have never heard with a benefits advisor. They can of the disease. also provide financial assistance You could provide them with in the form of grants – ask your a copy of this booklet or invite nurse in the hospital how to apply. them to download it from the As APL is a type of cancer, you Leukaemia Care website at www. will also be entitled to apply for leukaemiacare.org.uk a medical exemption certificate, It is important for you to know which means that you are entitled that people with any form of to free NHS prescriptions. Your GP cancer are covered by law by or clinical nurse specialist at the the Equality Act. This means hospital can provide you with the that legally your employer details of how to apply for this. If cannot discriminate against you are undergoing chemotherapy you and must make reasonable you may qualify for a Blue Badge arrangements and adjustments to help with hospital car parking. relating to your disease. To apply for a badge, contact your local council. If you would like general advice about some of the financial

Helpline freephone 08088 010 444 21 Talking about APL

Talking to your • Note information down to help haematologist you remember what was said. APL is a rare condition. It is • Be open when you discuss your important for you to develop a symptoms and how you are good working relationship with coping. Good patient-doctor your haematologist, so you are communication tends to given the best treatment possible. improve outcomes for patients.

The following gives advice Other tips: on working well with your • Bring someone along to your haematologist: appointment. They can provide • If it’s an initial consultation, support, ask questions and take take along a list of your current notes. medications and doses, and • Don’t be afraid to ask for a list of any allergies you may a second opinion – most have. haematologists are happy for • If you have a complicated you to ask. medical history, take a list of You need to tell your diagnoses, previous procedures and/or complications. haematologist if... You’re having any medical Make a list of questions to take • treatment or taking any products to your appointment. This will such as prescribed medicines, help the discussion with your over the counter treatments haematologist. or vitamins. It is important to • It can be useful to repeat back understand that treatments, what you have heard so that including complementary you can be sure that you fully therapies, which are perfectly safe understood. for most people, may not be safe if you are being treated for APL.

22 www.leukaemiacare.org.uk Remember, if you choose to start Talking to other people any form of complementary therapy outside of your medical Telling people you have a rare treatment, consult your condition like APL can be hard. haematology consultant or However, you might find it useful clinical nurse specialist prior to let your close family and to taking it. It is important friends, as well as your employer, to understand the difference know about your health condition. between complementary It might be easier to provide therapies, used alongside people with basic information and standard treatment, and give them information leaflets or alternative therapies, used booklets like this one about APL if instead of standard treatment. they want to know more in-depth There is no evidence that any form details. of alternative therapy can treat "I made a conscious decision to be APL. very open about my illness. Telling For help with talking to your family was tough. But I encouraged haematologist, you can access people to ask questions." more information about APL, It is probably best to focus including a section on ‘Questions conversations on the symptoms to ask your medical team’ at www. that you are experiencing, how leukaemiacare.org.uk/support- the condition affects you and and-information/information- how you feel about it. Often about-blood-cancer/blood- people misunderstand and, cancer-information/leukaemia/ unfortunately, it will mostly fall acute-promyelocytic-leukaemia to you to educate them as best which features a list of questions as you can. Where possible, it’s that you may want to ask. advisable to let people know what you find helpful and unhelpful, in terms of what others say and do. Often people make assumptions

Helpline freephone 08088 010 444 23 Talking about APL (cont.)

and do what they think helps. For reliable internet sources, example, saying you look well, charitable organisations or your recounting stories of others they consultant haematologist. The know with a similar diagnosis, more you know, the more you and encouraging you to look can share. ahead and stay positive isn’t Have a print out to hand always what you really want to • – It may help to have some hear. In many ways, the more information to hand to share you communicate with them the with family and friends. This will better. take the pressure off you having These points may help you: to remember everything they may want to know. • Explain that you have a condition that means your • Explain your needs – Try and bone marrow does not function be clear about what your needs properly, and this affects may be. Perhaps you need help the number of blood cells it with the weekly food shop, produces. help with cooking dinner, or someone to drive you to and Explain your symptoms (maybe • from appointments. You may you are tired, or have a lot of find that friends and family pain). are pleased that they can do • Explain what you need (maybe something to help you. more help day-to-day, or • Be open about how you feel – someone to talk to). Don’t be afraid of opening up You could also consider the about how you feel, as people following when telling people who care will want to help you about your diagnosis: as best they can. Talk as and when you feel comfortable, so • Find out more – Try to find those around you will know out as much as you can when you need them most. about your condition, from

24 www.leukaemiacare.org.uk Glossary

Acute Myeloid Leukaemia (AML) Chromosome A rapid and aggressive cancer X-shaped, thread-like structures of the myeloid cells in the bone which carry the genes, and are marrow. located in the nuclei of every cell in the body. There are 46 Anaemia chromosomes (23 pairs) in A condition where the number of human cells. red blood cells are reduced. Red blood cells contain haemoglobin Consolidation and transport oxygen to body A treatment phase given to cells. This may be due to a lack remove any leukaemia cells that of iron, leukaemia or sickle cell are still present after induction disease. treatment, as this will help reduce the risk of the leukaemia Autologous Stem Cell returning. Transplant A transplant of stem cells derived Fatigue from part of the same individual Tiredness and weakness receiving them. rendering the patient unable to work or perform usual activities. Bone Marrow A soft blood-forming tissue that Genes fills the cavities of bones and Genes are made up of DNA which contains fat, immature and stores the genetic information mature blood cells, including required to make human proteins. white blood cells, red blood cells and platelets. Induction The treatment phase intended to Chemotherapy kill the majority of the leukaemia Drugs that work in different ways cells in the blood and bone to stop the growth of cancer cells, marrow, and to restore normal either by killing the cells or by blood cell production. stopping them from dividing. Lymphoid Cell A cell originating in the bone

Helpline freephone 08088 010 444 25 Glossary (cont.)

marrow which will eventually Relapse Condition become lymphocytes and A relapse occurs when a patient antibodies. initially responds to treatment, Maintenance but after six months or more, the response stops. This is also An ongoing treatment phase sometimes called a recurrence. intended to prevent any leukaemia cells from recurring. Remission Lower doses of therapy are given Remission is said to have often for years. occurred when the blood cell counts have returned to normal and there are less than 5% of Low numbers of neutrophils, abnormal, immature leukaemia making you more vulnerable to cells still present in the bone infections. marrow. Complete remission is Neutrophils said to have occurred when there are no leukaemia cells anywhere White blood cells involved in else in the body. fighting inflammation and infection, specifically bacterial Stem Cells infections. The most basic cell in the body Platelets that has the ability to develop into any of the body’s specialised cell Small blood cells that help the types, from muscle cells to brain body form clots to stop bleeding. cells. Red Blood Cells White Blood Cells (or Small blood cells that contain Leukocytes) haemoglobin and carry oxygen The main role of white blood cells and other substances to all is creating an immune response tissues of the body. against both infectious disease Refractory Condition and foreign invaders. There are several different types of white A condition for which treatment blood cells, and each has a does not result in a remission. different role.

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

There are a number of helpful Bloodwise sources to support you during Bloodwise is the leading charity your diagnosis, treatment and into the research of blood cancers. beyond, including: They offer support to patients, • Your haematologist and their family and friends through healthcare team patient services. • Your family and friends 020 7504 2200 • Your psychologist (ask your www.bloodwise.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