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Plasma Cell Leukaemia (PCL)

A Guide for Patients Introduction

Being diagnosed with 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 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 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 . The distinction B- that have been between multiple myeloma and activated, and they produce a plasma cell leukaemia is based single type of which is on the presence of a circulating specific to a particular , peripheral 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 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- 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 overlap with multiple myeloma. in patients based on the types of 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. the type of leukaemia. ••M-protein is an antibody, also In PCL, immunophenotyping

8 www.leukaemiacare.org.uk identifies CD20 antigens more have a more favourable prognosis. commonly, and CD56 less MYC translocations are known to frequently. CD56 is negative in play a crucial role in progression about 80% of PCL patients, which of the PCL. The aggressive nature helps differentiate between PCL of primary PCL may partly be due and multiple myeloma. to the high numbers of MAF and MYC translocations, TP53 and Chromosome analysis KRAS mutations and inactivation Patients with PCL show a higher of TP53. incidence of high-risk genetic findings than patients with multiple myeloma. Chromosome analysis of primary PCL plasma cells shows abnormalities in at least 50% of patients. Mutations or translocations have been reported for t(11;14), t(14;16), t(11;14), t(14;20) and t(4;14), the KRAS genes, the tumour suppressor gene P53, as well as the cell regulator genes MYC and MAF, located on chromosomes 8 and 16, respectively. Translocation in genetics 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.

The translocation of the t(11;14) chromosome mutation indicates a poor prognosis in PCL patients, whereas, in multiple myeloma, patients with this abnormality

Helpline freephone 08088 010 444 9 Symptoms of PCL

Due to the low incidence of PCL, of platelets and red blood cells, details of its clinical presentation but high levels of white blood are relatively limited and mainly cells, which are mainly plasma based on a series of case reports. cells. Platelets are a type of blood cell that help to stop bleeding. The clinical presentation in patients with PCL typically involves symptoms due to the organ damage. These symptoms include renal failure and bone pain associated with softened area within the bones (called lytic bone lesions) resulting from hypercalcemia (raised calcium levels in the blood).

Other symptoms reported from case series include: ••Pale appearance due to anaemia ••Pleural effusion (fluid on the lungs) ••Enlargement of the lymph nodes, and liver

Rarely, the central nervous system is involved, where the leukaemia cells have spread to the brain and spinal cord. This involvement of organs outside the blood and bone marrow is common in primary PCL, particularly when compared with multiple myeloma.

Blood cell counts reveal low levels

10 www.leukaemiacare.org.uk Helpline freephone 08088 010 444 11 Diagnosis of PCL

PCL belongs to a unique subgroup abnormalities which is also seen of plasma cell abnormalities more frequently in primary PCL and has a different biological than with multiple myeloma. background, as well as distinct Despite having unique clinical and laboratory features, biological criteria, some of the compared with multiple myeloma immunological and genetic and the other plasma cell cancers. markers may overlap with Diagnosis of PCL requires more multiple myeloma. Therefore, the than 2000 circulating plasma diagnosis of PCL is based on the cells per millilitre of peripheral combination of the symptoms blood and/or a number of plasma experienced by the patient, cells representing more than 20% microscopic examination of of total white blood cells in the blood, lymph nodes or blood peripheral blood. marrow, and the findings from immunophenotyping and The diagnosis of primary PCL may chromosome analysis. be suggested by the presenting signs and symptoms which To confirm the diagnosis of a have a more rapid onset than patient with suspected PCL, in multiple myeloma, with a the following tests should be greater tendency of symptoms performed and reviewed carefully of increased metabolic rate such given the difficulty of diagnosis: as fevers, sweating, weight loss, Peripheral blood smear and fatigue. In addition, organ •• involvement, hypercalcaemia and ••Blood laboratory tests: Complete renal involvement are common blood count with differential, in primary PCL. Leukaemic electrolyte levels, creatinine, plasma cells are regularly found liver enzymes, bilirubin, in the liver, spleen and spinal alkaline phosphatase, lactate fluid in patients with PCL. In dehydrogenase, uric acid, β2 addition, central nervous system microglobulin involvement is associated with high-risk chromosome ••Bone marrow aspiration and

12 www.leukaemiacare.org.uk biopsy blood can be used to confirm the presence of the circulating cancer Serum protein electrophoresis •• plasma cells. Cancerous plasma with immunofixation. cells negative for CD56 are Electrophoresis is a diagnostic generally considered indicative of tool to visualise the fragments secondary PCL. of protein molecules Chromosome analysis of PCL Protein electrophoresis of a •• plasma cells shows mutations or sample from a 24-hour urine translocations of t(11;14), t(14;16), collection t(14;20) and t(4;14), KRAS genes, ••Magnetic resonance imaging tumour suppressor gene P53 and (MRI) scan and cerebrospinal regulator genes MYC and MAF. fluid examination in patients with neurological symptoms ••Immunophenotyping/ chromosome analysis

The diagnosis of PCL can easily be missed when using just light because the leukaemic plasma cells in the peripheral blood are very similar to circulating lymphocytes seen in conditions such as chronic lymphocytic leukaemia, hairy cell leukaemia, or marginal zone . Consequently, immunophenotyping and chromosome analysis are generally required.

Immunophenotyping of peripheral

Helpline freephone 08088 010 444 13 Prognosis of PCL

Considering the low incidence elevated β2-microglobulin, of primary PCL, data on its hypercalcemia, elevated serum prognosis are relatively limited. lactate dehydrogenase, low serum Overall survival of patients with albumin, advanced age and primary PCL is known to be very poor renal function. Additionally, poor when they are treated with PCL patients with lower platelet conventional chemotherapy, with counts and higher plasma cell the majority dying within a year of counts are known to have a poor the diagnosis. prognosis.

PCL is a very aggressive blood The way patients respond cancer with no standard to treatment is also a good treatment at present. indication of prognosis in primary Nevertheless, induction with PCL. If the PCL is resistant to a bortezomib-based regimen induction treatment, then their followed by an allogeneic stem prognosis is likely to be very poor. cell transplant or tandem stem Similarly, the failure of blood cell transplant is achieving plasma cells to decline by 50% promising results. Making the within 10 days, or to be cleared diagnosis early is crucially within four weeks, is thought important so as to be able to start indicative of unresponsive bortezomib-based chemotherapy disease and a negative prognostic as soon as possible followed by an factor. ASCT. This approach is known to In view of the poor prognosis prolong patient survival. for patients with primary PCL, Prognostic factors ground-breaking treatment regimens in addition to Patients with the t(11;14) bortezomib, that can be followed chromosome abnormality in by tandem ASCTs, are required primary PCL are known to have a to improve the prognosis for poor prognosis. patients. Negative prognostic factors for patients with PCL include

14 www.leukaemiacare.org.uk Helpline freephone 08088 010 444 15 Treating PCL

The ideal outcome of treatment survival. for patients with PCL is achieving Several chemotherapy regimens complete remission with have been tried with varying induction chemotherapy followed results as complete remission by a stem cell transplantation is difficult to achieve in many (SCT). patients with PCL. Conventional Complete remission is said to chemotherapy only achieves an have occurred when the following overall survival of between four conditions have been met: and 15 months, therefore new drugs and drug combinations are Blood cell counts return to •• being tested. These include the normal following treatments. ••Less than 5% of cancerous plasma cells are present in the Bortezomib bone marrow Bortezomib is a proteasome inhibitor which is used as a There are no cancerous plasma •• first-line treatment for newly cells anywhere else in the body diagnosed multiple myeloma Induction therapy with intensive patients and for the treatment chemotherapy and bortezomib- of relapsed/refractory multiple based regimens should be started myeloma and Mantle cell as soon as possible followed lymphoma. by an autologous stem cell In the prospective trial of transplantation (ASCT). bortezomib-based regimens in In most cases of PCL, the goal 29 patients with newly diagnosed of treatment is to reduce the primary PCL, bortezomib-based large number of plasma cells regimens were found to be very in the blood and bone marrow, effective and achieved an overall and prevent or reverse the survival of 18 months. complications in the liver, spleen A more recent retrospective and lungs. Successful treatment study of new drugs in 50 patients in these terms will help prolong

16 www.leukaemiacare.org.uk with primary PCL also showed Autologous stem cell that drug combinations which included bortezomib showed transplantation efficacy, decreased the rate of Given the poor prognosis of early mortality and also achieved patients with primary PCL, an an overall survival of 18 months. ASCT is nearly always used as a fundamental part of treatment Finally, a retrospective analysis for patients. Allogeneic stem cell of 10 patients with newly transplantation may be useful in diagnosed primary PCL who were younger patients. treated with a combination of bortezomib, cyclophosphamide In an ASCT, the patients’ own and dexamethasone followed by healthy stem cells are collected an ASCT, showed a response rate from the bone marrow before of 100% after induction therapy. they receive the high-dose Following the ASCT, after a chemotherapy. Following the 25-month follow up, survival rate chemotherapy treatment to was 70%. kill the cancerous plasma cells, the healthy stem cells Immunomodulators are transplanted back into the Results with immunomodulators patient. This prevents the stem such as lenalidomide or cells being damaged by the high- thalidomide for the treatment dose chemotherapy. of PCL have been mixed. In a Evidence of the benefits of ASCT prospective Phase 2 study of 23 was seen in a retrospective newly diagnosed primary PCL analysis of a case series of patients, lenalidomide combined patients with PCL, which showed with dexamethasone resulted in an increased median overall an overall survival rate of 63% survival of 34 months in patients after a median follow-up of 15 who received an ASCT, compared months. with 11 months for patients who did not.

The Centre for International Blood

Helpline freephone 08088 010 444 17 Treating PCL (cont.)

and Marrow Transplant Research Marrow Transplant Research, data in the United States analysed was also analysed for 50 patients data collected between 1995 and who received a single allogeneic 2006 for 97 patients with primary SCT. The three-year overall PCL who received an ASCT as part survival rate for these patients of their treatment. Overall survival was 39%. rate at three years was 64%. For patients who received a single Tandem stem cell ASCT, overall survival rate was transplantation 56% but for patients who received The tandem SCT treatment a tandem ASCT, overall survival approach leads to a significant rate rose to 84%. A tandem improvement in survival for ASCT means that two ASCTs are patients. Results with an performed within a period of no allogeneic SCT are poor compared more than six months apart. with tandem SCTs because they Allogeneic stem cell have a higher mortality rate following relapse. Therefore, an transplantation allogeneic SCT should only be In an allogeneic SCT, healthy stem performed if options are limited. cells from a matching donor, A large, recent, retrospective study which must at least be a partial of 117 patients with primary PCL match, are transplanted into the confirmed the treatment rationale patient. A sibling, parent or child of induction with a bortezomib- are likely to be good matches. based regimen followed by an Donors who are not relations may SCT with an overall survival of be found through national bone 35 months for patients who marrow registries. Allogeneic underwent an SCT and 13 months transplantations are generally for patients who did not. considered for younger patients. For patients not eligible for SCT- In the study mentioned by the based options, induction with a Centre for International Blood and bortezomib-based regimen should

18 www.leukaemiacare.org.uk be started and then followed with maintenance therapy to keep the PCL under control.

Despite a limited amount of evidence from prospective trials or retrospective studies, maintenance treatment following SCTs is increasingly recommended because of the high tendency for early relapse. Maintenance regimens may include either bortezomib or lenalidomide, or both, in combination-based regimens.

Helpline freephone 08088 010 444 19 Seeing your doctor

Your symptoms ••How long will it take to get the results back? Whatever symptoms you have, make sure you write a list of all ••How common is my condition? of them to share with your doctor What sort of treatment will I as they may be important to your •• need? treatment. ••How long will my treatment Your appointment last? Arranging an appointment with How will I know if my treatment your GP will be one of the first •• has worked? things you will need to do when you start to notice symptoms. ••What will the side effects be? Pick a time convenient for you that you know you will be able to ••Are there any foods or attend. medications I need to avoid? Your preparation ••Will I be able to go back to work? It is important to know exactly ••Where can I get help with what you would like to ask claiming benefits and grants? your doctor. Make a list of your ••Where can I get help dealing questions and leave spaces for with my feelings? the answers so you can write them down when you see the Talking to your doctor doctor. This way you can go into Be honest with your doctors; there the appointment ready and is no need to feel embarrassed prepared. about anything. If you saw your Examples of questions to ask the healthcare team before seeing doctor: your doctor, be sure to share with your doctor everything your ••What tests will I need to have? healthcare team told you about ••What will the tests show? your condition, the blood tests

20 www.leukaemiacare.org.uk you had performed, and the next to you as this may be able to help steps in your PCL journey. Ask also you with your feelings towards if you will receive any intensive your diagnosis and treatment. treatment or palliative care. Your support If it helps, take a family member or friend in with you for support. Some people take a pen and paper in to make notes and repeat back to their doctor everything they have been told to ensure that they are on the same page, and that nothing has been missed or forgotten. The next steps Always ensure that you leave the GP surgery, or the hospital, having shared everything you know about your condition, with all of your questions answered, and knowing exactly what the next steps are, whether it is more tests, further treatment or palliative care. You can ask for a summary letter of the consultation to have everything in writing. Your doctor will generally send a letter like this to your GP.

Furthermore, be sure to access all of the other support available

Helpline freephone 08088 010 444 21 Telling your family

Planning who to tell help keep both yourself and the other person calm. Deliver what Telling your family and friends you have to say slowly, calmly, that you have been diagnosed concisely, and sentence by with PCL can be difficult. sentence to allow the other person You may want to create a list of time to take in the information. people you want to tell, starting Be sincere and hold their hands if with close family and friends, and you need to. then extending it beyond, from You can use the following your colleagues at work to friends sentences to help you articulate in your neighbourhood. what you need to say: Planning what to say ••"This is going to be difficult, but It is important to know what I need to tell you something." you want to say and exactly how ••"I’ve had some bad news but much you want people to know. there’s a good chance that Being clear in your mind about everything will be okay after I’ve that before speaking to anyone had treatment." will make this a much smoother experience. Know your story that ••"You know I’ve been feeling you want to tell, the diagnosis, unwell for a while. I’ve had some the prognosis, the next treatment tests and they’ve found out steps, and what you expect to what’s wrong." be going through physically and emotionally. Be sure to speak to How to respond people in an environment where Naturally people will feel sad and both of you can hear each other concerned for you. Everyone deals clearly and where there are likely with this type of news in their own to be no interruptions. way, from shock and silence, to questions and support. Invariably, How to say it people respond positively, which Using a conciliatory tone will in turn means you will respond back positively.

22 www.leukaemiacare.org.uk Accepting help Sometimes people feel guilty for You can receive help getting cancer, that they weren’t from us on how to strong enough, and that they deal with telling your will be a burden on those around them. This is where your loved family and friends. ones come in, so make sure you You can visit www. do ask for and accept offers to leukaemiacare.org. help and support you. Do not try to uk, or call 08088 cope on your own. If they offer to 010 444, to find out help, tell them that you will get in touch when you need them. more.

Repeating yourself to different people can become burdensome. Your network of family and friends can help you out by telling those beyond them about your current situation.

Helpline freephone 08088 010 444 23 Managing your emotions

Being told that you have cancer and support will be available to may be difficult for you to deal you from your healthcare team, with. and you will have access to counsellors and therapists if and Indeed, you may have a positive when you need it. demeanour, which will obviously be helpful to you during the next Isolation steps in the management of your condition. However, you may If you have received a diagnosis of experience a range of emotions, PCL, you may feel alone. including uncertainty, isolation, Alternatively, you may feel dealing anxiety, anger, sadness and with your cancer allows you to be depression. Understanding each around those closest to you. Being emotion and developing ways that around those closest to you, such help you deal with them will help as your family and friends, can be you move forward with your life. positive and negative. Uncertainty Let them know what you do and don’t want to do, how you do You may think "What happens and don’t wish to be treated, next?". You may be unsure about and what you do and don’t feel your health and what the future comfortable talking about. holds for you. You may or may Sometimes, it is difficult for your not have had meetings with your family, friends and colleagues healthcare team to discuss the to understand what you are next steps. Once you have a clear feeling and going through. Being path set out in front of you, you clear will help create the kind of will be able to develop a clearer positive, supportive, and caring picture of where you are headed. environment that will help as you Gaining a sensible balance move forward with your life. between being vigilant about your symptoms and carrying on Anxiety with your life will help ease any anxieties. Help, care, kindness Being fearful of the unknown, especially when we are feeling

24 www.leukaemiacare.org.uk threatened, is natural. You may Understanding exactly what is experience an increased heart making you angry will help you rate, rapid breathing, and muscle deal with your feelings effectively. tension. These things help us to In addition, setting yourself face a danger or run away. These achievable goals that stretch you changes in you are part of the will help reduce the anger and ‘fight or flight’ response. Any impatience you feel, especially feeling of discomfort, pain or even with each passing success. Don’t another appointment with your forget to congratulate yourself for healthcare team may elicit such each successfully completed task, responses and give you sleepless however small. nights or feelings of worry. This is Physical exercise is a great completely natural. way to release your anger and Such reflexes and responses will frustrations, and channel your ease over time with the building energy positively with no negative of daily routines and planning impact on your body. Talking things for the future, which will about your feelings and letting help you to cope with the physical them out will also help stop you effects of anxiety. Cognitive lashing out at people and keep behavioural therapy can help you you calm. deal with your worrying thoughts. Sadness and Anger depression Feeling angry after your diagnosis You may feel a sense of loss of is natural and normal. You may the person you used to be, and be angry with yourself, your body, how safe you felt. You may also with the healthcare team or feel that your illness is a heavy with family and friends. You may burden on those around you. You display your anger as impatience, might be feeling low, which is irritability and frustration with a natural effect of your illness, people and things that would not treatment and recovery. However, normally bother you. if this low mood persists for more

Helpline freephone 08088 010 444 25 Managing your emotions (cont.)

than several weeks, and you feel your appearance and how you hopeless, and lose interest and feel emotionally. In turn, this can pleasure with things in life, then knock your self-confidence and you may have depression. self-esteem. Your feelings of relief, hope, and optimism have just Your first steps should be to been replaced with their polar speak to your loved ones around opposites. you about your mood and state of mind, and then contact your You can gradually build your self- GP. You may lift the way you feel confidence and self-esteem back by engaging in activities that up by engaging in the activities you were enjoying before your you did before your diagnosis, and diagnosis, to connect back with socialising with family, friends, your life. Only do as much as and fellow patients. This will help can and try and talk about your create a supportive atmosphere to thoughts and feelings. This will get you back to your old self. help lighten your burden and put things into perspective. If you Mindfulness and have made any acquaintances or relaxation friends in the same position as Simple practices from you, talk to them over coffee as mindfulness and relaxation they will understand what you are techniques can help you calm the facing. mind, release tension and ease Self-confidence any pain in your muscles. Being forced to adjust from your ••Put yourself in a relaxing daily routine during the visits to environment, sitting or lying the hospital for treatment can down comfortably. take its toll. This interruption of Loosen your clothing so you can your life, along with your lack of •• move more freely. energy because of your condition and the effects of your treatment, ••Calmly breathe in through your can impact on how you feel about nose, and out through your

26 www.leukaemiacare.org.uk mouth, developing a steady natural rhythm, focusing on your chest and abdomen as you do so. ••Visualise that you are inhaling positivity and exhaling negativity.

By taking some time out of your day to do these exercises, you can help quieten your mind and remove the stress of coming to terms with your diagnosis, so you feel calmer and more relaxed.

Helpline freephone 08088 010 444 27 Survivorship

Someone who is living with or is ends, so you may feel a mixture beyond a cancer diagnosis can be of emotions from relief to fear, considered a cancer survivor. anxiety and uncertainty about the future. You may wonder how you Survivorship can be defined as: will slot back into your life after "...cover[ing] the physical, coming through the treatment psychosocial and economic period. issues of cancer, from diagnosis Your survivorship pathway began until the end of life. It focuses at the point when you were on the health and life of a diagnosed with PCL. By this point, person with cancer beyond the you will have been starting to diagnosis and treatment phases. receive support for work, finance, Survivorship includes issues and personal relationships related to the ability to get health through to managing pain, fatigue care and follow-up treatment, late and making positive lifestyle effects of treatment, secondary changes, such as starting a cancers and quality of life. Family healthy diet and gentle exercising. members, friends and caregivers are also part of the survivorship Your individual needs as a patient experience." will be identified and addressed, including: When living with cancer, you will face new challenges to cope with ••Dealing with the emotional from physical to psychological impact of receiving a PCL and social ones. Survivorship diagnosis which may have aims to provide personalised care created feelings of uncertainty, based on your need to improve fears of recurrence and your health, wellbeing, quality difficulties in planning for the of life, and your confidence and future. These will be discussed motivation, to help you manage. with you to develop your Survivorship also focuses on your individualised care plan with health and life with cancer after support from social care staff the end of treatment until the end and therapists, as you need it. of life. At this point, your routine Improving your quality of life of meeting frequently with your •• through efficient and co- healthcare professionals also

28 www.leukaemiacare.org.uk ordinated care during your physical equipment and taught treatment, with effective about various coping strategies communication within the to adapt to your new situation. treatment team, and a positive Supporting you with advice attitude. •• for social and financial ••Taking care of any comorbidities difficulties, including caring – that is, other medical responsibilities, your inability to conditions and diseases participate in social activities, – and offering you cancer any debt and financial worries rehabilitation based on your from not being able to work, and clinical needs as assessed by perhaps the need to return to informed professionals and work before you feel ready. ensuring compliance with the Receiving health and nutrition National Cancer Rehabilitation •• advice from a nutritionist Pathways and Rehabilitation on following a healthy and Peer Review requirements. balanced diet to help improve ••Providing you with a treatment your general health and summary from the diagnosis of wellbeing. The World Cancer your condition to the end of your Research Fund published a treatment. This would include report for cancer survivors any ongoing medication and which suggests that even small noting possible symptoms that dietary and lifestyle changes may occur in the future. You can produce large health would also be provided details benefits. of who to contact in addition to your GP for any concerns you may have. ••Preparing you fully for the impact of your PCL and treatment, the physical and physiological side effects of treatments and the psychological impact of PCL in general. You will be provided

Helpline freephone 08088 010 444 29 Palliative care

Palliative care, also known as religious leaders, if you would like supportive care, involves a holistic this. Your palliative care services or "whole person" approach, which may be provided by the NHS, local includes the management of your council or a charity. You may pain and symptoms as well as receive day-to-day care at your psychological, social and spiritual home and at the hospital. support for you and your loved ones. What is the clinical course? Palliative care aims to reduce your symptoms, control your PCL, You will have a number of extend your survival, and give treatments and be prone to you and your loved ones the best frequent infections because of quality of life possible. Your doctor the PCL and the impact of your will discuss the options with you treatments. Your therapy may in detail before you decide the continue because of potential next steps. remission and/or useful palliation. Who provides palliative You may experience various pains care? and other clinical complications Your palliative care will be such as: provided by a team of health and Bone pain: Radiotherapy and/ social care professionals trained •• or oral steroids, and sometimes in palliative medicine who will non-steroidal anti-inflammatory coordinate your care. drugs (NSAIDs), may be used, These professionals can include although these are used with your GP, hospital doctors and caution because they can nurses, community nurses, interfere with your immune hospice staff and counsellors, system and kidney function. social care staff, physiotherapists, Bone marrow failure: Blood occupational therapists, •• and platelet transfusions complementary therapists, and are provided to prevent and

30 www.leukaemiacare.org.uk fight recurrent infections and Treatments can include bleeding episodes. analgesics, antidepressants and/or anticonvulsant Oral problems: Analgesic mouth •• medication used in tandem washes and topical ointments with opioids. may help with ulceration. Chewing gum, and mouth ••Hypercalcaemia: Treatment washes, have been shown to is usually with intravenous help with dry mouth, tooth hydration and intravenous decay and oral thrush. bisphosphonates. ••Night sweats and fever: These ••Loss of appetite: Low-dose can place a heavy burden on steroids may temporarily carers because of so many boost the appetite, while small, changes of night clothes and frequent and appetising meals bedding. and supplement drinks will also help. ••Pathological fractures: Orthopaedic intervention and subsequent radiotherapy, with consideration given to prophylactic pinning of long bones and/or radiotherapy to prevent fractures will be performed. This will reduce the likelihood of complex pain syndromes developing. ••Spinal cord compression: Immediate high single daily dose oral steroids will be given. ••Back pain from wedge and crush fractures of the vertebrae of the spinal column:

Helpline freephone 08088 010 444 31 End of life care

When does end of life Who provides end of care begin? life care? If your treatment hasn’t worked A team of health and social care and you are going through professionals may be involved palliative care, you may be offered in your end of life care, including end of life care. End of life care hospital doctors and nurses, your begins when you need it and may GP, community nurses, hospice last a few days, months or years. staff and counsellors, social care staff, physiotherapists, What does end of life occupational therapists or care involve? complementary therapists, and religious leaders, if you would like End of life care is support for this. If you are being cared for at people who are in the last few home or in a care home, your GP months or years of their life. The will have overall responsibility for aim is to help you enjoy a good your care with the support from quality of life until you die, and to community nurses, along with die with dignity. The professionals your family and friends. looking after you will ask you about your wishes and What choices do I have preferences on how to be cared for and put these into action. They in terms of end of life will also provide support to your care? family, carers and loved ones. You Deciding where you want to die will be able to decide where you can be a difficult choice to make. will receive end of life care, be it at Working out what you and your home or in a care home, hospice loved ones want, together with or hospital. The same will be true seeing what services are available of where you would like to die. to you, can help to make the Wherever you are, you will receive decision a little easier. high quality end of life care. ••Staying at home: A place of familiarity, surrounded by your

32 www.leukaemiacare.org.uk loved ones, may be something be used to staying in a hospital that you will find reassuring. ward, the care routine cannot External care professionals will always be tailored to your be able to visit you at home to specific needs. Pressures on make sure your symptoms are the NHS mean that your stay looked after. will only be as long as strictly required. As soon as the Hospices: These are specialised •• condition you were admitted in looking after those with life- for has been resolved, you will limiting illnesses and those need to go back to your home who are coming to the end of or nursing home. However, a their life. Hospices are staffed number of specialists will be with care professionals who available to help look after you are able to keep an eye on you, for specific problems, and a make sure that your symptoms number of hospitals also have a are controlled and offer you a designated palliative care team number of services to make for patients who require them. your stay as comfortable as possible. For more information Whatever your choice, speak on the care that they can with your GP or healthcare team provide, go to https://www. who will be able to help you put hospiceuk.org/ everything into place. ••Residential care/nursing homes: If you think that your stay may be a few months or more, then a nursing home may be more suitable than a hospice. These can be private or run by a charity or the local council so be sure to check if there are any fees. ••Hospitals: Although you may

Helpline freephone 08088 010 444 33 Glossary

Albumin and transport oxygen to body cells. This may be due to a lack Albumin is a protein made by of iron, leukaemia, or sickle cell the liver which accounts for up disease. to 60% of the total protein in the blood. Albumin has many roles Antibody which include preventing fluid A large Y-shaped protein produced from leaking out of the blood by B-cell lymphocytes in response vessels, nourishing tissues, and to a specific antigen, such as transporting hormones, vitamins, a bacteria, virus, or a foreign drugs and calcium throughout the substance in the blood. The body. Levels of blood albumin can antibodies neutralise the bacteria be decreased because of a liver and viruses. disorder or kidney disease. Antigen Alkaline phosphatase A toxin or other foreign substance An enzyme found in various which induces an immune tissues throughout the body, with response in the body, especially the highest concentrations being the production of antibodies. found in liver or bone. Raised levels of alkaline phosphatase β2 microglobulin (beta 2 in the blood are most commonly microglobulin) caused by liver disease or bone A protein found on the surface disorders. of most cells in the body and Amino acids which is shed into the blood, particularly by B lymphocytes and Organic molecules which are tumour cells. It is used to assess the building blocks for making the severity of certain cancers, . including multiple myeloma and Anaemia some . A condition where the number of Bilirubin red blood cells are reduced. Red The breakdown product of red blood cells contain haemoglobin blood cells.

34 www.leukaemiacare.org.uk Bone marrow failure conditions have been met: The term used when the bone ••Blood cell counts return to marrow is unable to keep up with normal the body’s need for white and red blood cells and platelets. ••Less than 5% of blasts (abnormal, immature, early Central nervous system cells) are still present in the A part of the nervous system bone marrow which includes the brain and ••There are no blast/cancer cells spinal cord. anywhere else in the body Chemotherapy Cytogenetic Drugs that work in different ways Relating to the study of to stop the growth of cancer cells, inheritance in connection with either by killing the cells or by the structure and function of stopping them from dividing. chromosomes. Chromosomes Electrolytes Thread-like structures which carry Salts and minerals in the blood the genes and are located in the that help conduct electrical nuclei of every cell in the body. impulses in the body. They include There are 46 chromosomes (23 sodium, potassium, chloride and pairs) in humans. bicarbonate among others. ClinicalTrials.gov Electrophoresis ClinicalTrials.gov is a database A diagnostic tool to visualise of trials and includes details of fragments of protein molecules. approximately 276,190 research studies in 205 countries. Fatigue Complete remission Tiredness and weakness rendering the patient unable to Complete remission is said to work or perform usual activities. have occurred when the following

Helpline freephone 08088 010 444 35 Glossary (cont.)

Genes Lymph nodes Genes are made up of DNA which Components of the lymphatic stores the genetic information system (part of the body’s required to make human proteins. ) that contain the lymphocytes which produce Hypercalcemia antibodies and to A condition in which calcium digest dead cells. Lymph nodes levels in the blood are increased. are swollen with cell fragments in This can result in weakening of the event of infection or cancer. the bones, formation of kidney They are located mainly in the stones, and abnormalities of heart neck, armpit and groin. or brain function. Lymphocytes Immunoglobulins Lymphocytes are a type of Immunoglobulins, also known that are vitally as antibodies, are proteins important to the immune produced by the plasma cells that response. There are three types of act by recognising and binding lymphocytes: B-cells, T-cells and to particular antigens, such natural killer (NK)-cells. B-cells as bacteria or viruses, thereby produce antibodies that seek helping in their destruction. out invading organisms. T-cells destroy the organisms that have Lactate dehydrogenase been labelled by the B-cells, as An enzyme that is required during well as internal cells that have the process of turning glucose become cancerous. NK-cells (sugar) into energy for body attack cancer cells and viruses. cells. Lactate dehydrogenase is released during tissue damage, Multiple myeloma and it is therefore a useful marker A common blood cancer arising for common injuries and disease from plasma cells. It accounts for such as heart failure or muscle 15% of blood cancers, and 2% of all injury. cancers.

36 www.leukaemiacare.org.uk Neoplasm analysed to attempt to provide an answer. A medical term for cancer, meaning literally a new and Radiation treatment abnormal growth of tissue Cancer treatment that uses high anywhere in the body. doses of radiation to kill cancer Plasma cell cells and shrink tumours. A type of white blood cell that Refractory multiple myeloma produces antibodies and is Multiple myeloma in which derived from a B-cell . treatment does not result in a It is an ovoid (egg-shaped) cell remission, or that gets worse with an off-centre nucleus. within six months of the last Platelets treatment. However, the multiple myeloma may be stable. One of the types of blood cells that help to stop bleeding. Relapsed multiple myeloma Prognosis A relapse occurs when a patient initially responds to treatment for An indication of how well a multiple myeloma but, after six patient is expected to respond months or more, response stops. to treatment based on their This is also sometimes called a individual characteristics at recurrence. the time of diagnosis or other timepoints during the condition. Retrospective study Prospective study Study which analyses data from completed studies where A study designed and planned the results are already known. to determine a specific answer Retrospective analysis looks back or aim; for example, whether at existing data that has already treatment A is better than been recorded to determine, for treatment B. The study will be example, the characteristics of conducted in patients who meet a disease, the efficacy or safety particular inclusion criteria, and of a treatment, or factors which the results are collected and

Helpline freephone 08088 010 444 37 Glossary (cont.)

might have led to a disease. Retrospective analyses are the opposite of prospective studies. Translocation In genetics, 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. Uric acid A product of the metabolic breakdown of purine nucleotides which are the chemical building blocks of DNA. Uric acid is a normal component of urine.

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38 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 39 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.

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

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