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Acute Promyelocytic Leukaemia (APL) 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 blood 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 Platelets 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 bone marrow. The term shortest-lived white cells are acute indicates that the called neutrophils. 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 white blood cell • 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.
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