Plasma Cell Leukaemia (PCL) A Guide for Patients Introduction Being diagnosed with Plasma Cell Leukaemia (PCL) can be a shock, particularly when you may never have heard of it. If you have questions about PCL – 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. For more information, talk to your ensures it is of the highest quality. haematologist, clinical nurse Unfortunately, due to the rarity of specialist or hospital pharmacist. PCL, we were unable to complete the production process which meant Booklet compiled by our Patient that this booklet cannot be formally Information Writer Isabelle Leach accredited. However, we assure you and peer reviewed by Dr Salim that this information was created Shafeek. with the same values as that which is. Disclaimer: As we are accredited by the Information Standard, all of our information has to adhere to a standardised process that If you would like any information on the sources used for this booklet, please email [email protected] for a list of references. Version 1 Printed: 06/2019 2 www.leukaemiacare.org.uk Review date: 06/2021 In this booklet Introduction 2 In this booklet 3 About Leukaemia Care 4 What is PCL? 6 Symptoms of PCL 10 Diagnosis of PCL 12 Prognosis of PCL 14 Treating PCL 16 Seeing your doctor 20 Telling your family 22 Managing your emotions 24 Survivorship 28 Palliative care 30 End of life care 32 Glossary 34 Useful contacts and further support 39 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 – 5:30pm 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 – 5:30pm. 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 quarterly 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 PCL? Plasma cell leukaemia (PCL) is for 1% of previously diagnosed a rare and aggressive variant of multiple myeloma cases and is a multiple myeloma characterised more aggressive disease with a by a very high number of poorer prognosis. plasma cells in the blood and Plasma cells develop from bone marrow. The distinction B-lymphocytes that have been between multiple myeloma and activated, and they produce a plasma cell leukaemia is based single type of antibody which is on the presence of a circulating specific to a particular antigen, peripheral blood plasma cell for example the E coli bacteria. count of greater than 20% of white blood cells and/or more than Who is affected by 2x106/ml of leukaemic plasma cells in the peripheral blood. plasma cell leukaemia? Because PCL is such a rare Approximately 60 to 70% of PCL condition, information about its cases are new blood cancers at incidence, disease characteristics diagnosis, known as primary and treatment have mainly been PCL, while 30 to 40% occur as gathered retrospectively from secondary transformations of case reports or series of case multiple myeloma in patients reports. There have not been many with relapsed/refractory multiple prospective trials of patients with myeloma, when they are known as PCL. secondary PCL. Retrospective studies have Primary PCL is a distinct cancer provided most of the information with different molecular and that is known about PCL. This chromosomal findings than is where data from studies that secondary PCL and multiple have already been completed are myeloma. Primary PCL represents subsequently analysed to see 2 to 4% of myelomas. Multiple which treatments achieved the myeloma is a relatively best results for which patients. common cancer of plasma cells representing 10 to 15% of all blood A prospective trial is designed cancers. Secondary PCL accounts to determine a specific answer, 6 www.leukaemiacare.org.uk for example what are the disease a retrospective study of the characteristics of a condition, incidence of haematological or whether treatment A is cancers from cancer registries better than treatment B for the for the years 2000 to 2002, that condition. The study is conducted included 92 cases of PCL. in patients who meet particular Based on data from a series of inclusion criteria and following patient case reports, the median completion of the trial, the results age of patients with primary PCL are collected and analysed. Until at diagnosis is between 50 and recently, assessing treatments for 59 years. This is younger than for PCL using prospective studies was multiple myeloma patients where not possible as it was difficult the median age at diagnosis to recruit enough patients for is between 66 and 70 years, a trial with meaningful results. confirming that primary PCL is The largest prospective trial to a different condition to multiple date included 29 patients with myeloma. PCL is twice as common newly diagnosed primary PCL to in people of African descent investigate the efficacy of drug compared with Caucasians, and combinations which included it is slightly more common in bortezomib. See the treatment men than women (60% to 40%); section for more details about however, this data is based on this trial. case series. In a more recent The risk of suffering from PCL retrospective study of 50 patients is very low with an estimated with primary PCL, the male to incidence of 0.4 cases per female ratio was 50% to 50%. million individuals per year. Secondary PCL occurs after the This incidence is from a recent initial diagnosis of myeloma retrospective study of case and gradual genetic progression reports where 117 patients with and changes. This can be primary PCL were treated between during the natural history of the January 2006 and December myeloma progression through 2016. A similar incidence for various treatments and new PCL was previously found in mutations at various time Helpline freephone 08088 010 444 7 What is PCL? (cont.) points like a subsequent relapse. called an immunoglobulin, The likelihood for developing which is secreted by cancerous secondary PCL is very low at about plasma cells and can be 0.5 - 1%. There are no predictive detected in the blood and/or the factors or features towards urine of most myeloma patients. secondary PCL, but the average Multiple myeloma is age of diagnosis for secondary • classified depending on which PCL is 66 years old. immunoglobin antibody is What causes plasma identified. M-protein will cell leukaemia? normally be an immunoglobin G (IgG) or an immunoglobin A The causes of primary PCL are (IgA). Less commonly, IgD or IgE currently unclear. The control are seen. Secondary PCL occurs mechanisms that keep the more frequently in IgE and IgD plasma cells within the bone myelomas. marrow, rather than letting them enter the blood stream as in PCL, • These are areas of overlap are still unknown. between the secretory type of PCL and multiple myeloma. Primary PCL is a unique subgroup of the plasma cell myelomas, and In non-secretory type of PCL, no it displays a different biological M-protein is secreted. background and separate Immunophenotyping laboratory features. Despite having unique biological criteria, The immunophenotyping process some of the genetic markers may helps analyse the antibodies overlap with multiple myeloma. in patients based on the types of antigens or markers on the PCL has two types: secretory and surface of the cancer cells. non-secretory. According to which antibodies are In the secretory type of PCL, present, it is possible to identify M-protein is released.