Disabled, invisible and dismissed-The lived experience of fatigue in people with myeloproliferative neoplasms.

BACKGROUND: Myeloproliferative neoplasms (MPNs) are rare haematological cancers. Several studies report the most common MPN symptom leading to reduced quality of life is fatigue. Yet, how fatigue affects the lives of people with MPN is not well described. AIMS: The purpose of this qualitative study is to better understand the lived experience of fatigue associated with MPN. METHODS AND RESULTS: People with MPN who had experienced fatigue were invited to complete an online survey and if eligible, then to participate in semi-structured interviews and focus groups, exploring their experiences of fatigue. Thematic analysis of interview transcripts by two researchers produced themes describing the lived experience of fatigue. Twenty-three people with MPN participated in seven interviews and four focus groups. Qualitative data revealed how fatigue significantly affected participants' experiences of functional, social, family and emotional wellbeing. Participants reported that fatigue was infrequently acknowledged or addressed by health professionals, and a lack of information or support to manage their fatigue. Four themes including 12 sub-themes describe the experience of fatigue in MPN: (1) the distress of the MPN diagnosis, (2) sensations of fatigue, (3) daily life and emotional burden with fatigue and (4) how people managed their fatigue with limited guidance. CONCLUSION: Fatigue in MPN is common, debilitating and distressing. It affects all aspects of health, wellbeing and life. Health professionals could affect patients' lives substantially by acknowledging and understanding fatigue in MPN, including contributing factors and potential opportunities for management. More systematic data describing the causes and management of MPN fatigue is needed.

Tobacco use in the Myeloproliferative neoplasms: symptom burden, patient opinions, and care.

BACKGROUND: Patients with Philadelphia-negative Myeloproliferative Neoplasms (MPN) suffer from numerous symptoms and decreased quality of life. Smoking is associated with an increased symptom burden in several malignancies. The aim of this study was to analyze the association between smoking and MPN-related symptom burden and explore MPN patients' opinions on smoking. METHODS: A total of 435 patients with MPN participated in a cross-sectional internet-based survey developed by the Mayo Clinic and the Myeloproliferative Neoplasm Quality of Life Group. Patients reported their demographics, disease characteristics, tobacco use, and opinions on tobacco use. In addition, MPN-related symptoms were reported via the validated 10-item version of the Myeloproliferative Neoplasms Symptom Assessment Form. RESULTS: Current/former smokers reported worse fatigue (mean severity 5.6 vs. 5.0, p = 0.02) and inactivity (mean severity 4.0 vs. 3.4, p = 0.03) than never smokers. Moreover, current/former smokers more frequently experienced early satiety (68.5% vs. 58.3%, p = 0.03), inactivity (79.9% vs. 71.1%, p = 0.04), and concentration difficulties (82.1% vs. 73.1%, p = 0.04). Although not significant, a higher total symptom burden was observed for current/former smokers (mean 30.4 vs. 27.0, p = 0.07). Accordingly, overall quality of life was significantly better among never smokers than current/former smokers (mean 3.5 vs. 3.9, p = 0.03). Only 43.2% of the current/former smokers reported having discussed tobacco use with their physician, and 17.5% did not believe smoking increased the risk of thrombosis. CONCLUSION: The current study suggests that smoking may be associated with increased prevalence and severity of MPN symptoms and underscores the need to enhance patient education and address tobacco use in the care of MPN patients.

Recruitment strategies and geographic representativeness for patient survey studies in rare diseases: Experience from the living with myeloproliferative neoplasms patient survey.

BACKGROUND: Recruitment of individuals with rare diseases for studies of real-world patient-reported outcomes is limited by small base populations. Myeloproliferative neoplasms (MPNs) are a group of rare, chronic, hematologic malignancies. In this study, recruitment strategies and geographic representativeness from the Living with MPNs survey are reported. METHODS: The Living with MPNs online cross-sectional survey was conducted between April and November 2016. Individuals 18 to 70 years of age living in the United States and diagnosed with an MPN were eligible to participate. Recruitment approaches included direct contact via emails and postcards; posts on MPN-focused social media and patient advocacy websites; postcard mailings to doctors' offices; and advertisements on medical websites, Google, and Facebook. Geographic representativeness was assessed based on the number of survey respondents living in each state or the District of Columbia and by the number of survey respondents per 10 million residents. RESULTS: A total of 904 respondents with MPNs completed the survey. The recruitment method yielding the greatest number of respondents was advertisements on MPN-focused social media (47.6% of respondents), followed by emails (35.1%) and postcards (13.9%) sent through MPN advocacy groups. Home state information was provided by 775 respondents from 46 states (range of respondents per state, 1-89). The number of respondents per 10 million residents in the 46 states with respondents ranged from 12.1 to 52.7. CONCLUSIONS: Recruitment using social media and communications through patient groups and advocacy organizations are effective in obtaining geographically representative samples of individuals with MPNs in the United States. These approaches may also be effective in other rare diseases.

The SIMM study: Survey of integrative medicine in myeloproliferative neoplasms.

Myeloproliferative neoplasms (MPNs) are characterized by significant symptom burden. Integrative medicine (IM) offers unique symptom management strategies. This study describes IM interventions utilized by MPN patients and the association with symptom burden, quality of life, depression, and fatigue adjusted for lifestyle confounders. MPN patients were surveyed online for IM utilization, MPN symptom burden (MPN-Symptom Assessment Form Total Symptom Score), depression (Patient Health Questionnaire), fatigue (Brief Fatigue Inventory), and a single question on overall quality of life. Measures were compared by IM participation and adjusted for alcohol and tobacco use, BMI, diet, and MPN type using multiple linear and logistic regression. A total of 858 participants were included in the analysis. Aerobic activity (p =< 0.001) and strength training (p = 0.01) were associated with lower mean symptom burden while massage (p =< 0.001) and support groups (p =< 0.001) were associated with higher levels of symptom burden. Higher quality of life was reported in massage (p = 0.04) and support groups (p = 0.002) while lower quality of life was noted in aerobic activity (p =< 0.001) and strength training (p = 0.001). A lower depression screening score was noted in those participating in aerobic activity (p = 0.006), yoga (p = 0.03), and strength training (p = 0.02). Lower fatigue was noted in those participating in aerobic activity (p =< 0.001) and strength training (p = 0.03) while higher fatigue was noted in those participating in massage (p =< 0.001) and breathing techniques (p = 0.02). Data available on request from the authors. This international survey of MPN patients on IM usage, has shown that patients who participated in a form of IM had a pattern of decreased levels of symptom burden, fatigue, depression, and higher QoL, as adjusted for health lifestyle practices overall.

Depressive symptoms and myeloproliferative neoplasms: Understanding the confounding factor in a complex condition.

BACKGROUND: Philadelphia chromosome negative myeloproliferative neoplasms (MPNs), including essential thrombocythemia, polycythemia vera, and myelofibrosis, have severe function-limiting symptom burden that is experienced by the majority of patients. Previous studies have suggested that depression may be present in over a quarter of MPN patients, but to date no studies have evaluated the relationship between depression and other variables such as symptoms. METHODS: A 70-item internet based survey regarding fatigue and mood symptoms was developed by a multidisciplinary team of MPN investigators, patients and patient advocates including Patient Health Questionnaire and the Myeloproliferative Neoplasm Symptom Assessment Form was completed by over 1300 patients with MPN diagnosis. RESULTS: There were 309 respondents (23%) with PHQ-2 scores ≥ 3. In this analysis, we found worse systemic symptom burden in individuals reporting depressive symptoms. CONCLUSION: This analysis suggests the importance of depression in contributing to as well as confounding symptomatology in MPN patients, and suggests that this critical variable should also be addressed by clinicians and researchers alike when comprehensively assessing symptom burden etiologies.

The Myeloproliferative Neoplasm Landscape: A Patient's Eye View.

Patients with myeloproliferative neoplasms (MPNs), a group of rare haematological conditions including polycythaemia vera, essential thrombocythaemia, and myelofibrosis, often experience a range of symptoms which can significantly impact their quality of life (QoL). Although symptom burden is highest in myelofibrosis and high-risk patients, lower-risk patients also report symptoms impacting their daily life and ability to work. In addition to physical symptoms, MPNs affect emotional well-being, with anxiety and depression frequently reported by patients. Despite significant advances in treatment options, such as the introduction of JAK1/JAK2 inhibitors, therapy for MPNs is often palliative; therefore, reduction of symptoms and improvement of QoL should be considered as major treatment goals. One of the main issues impacting MPN treatment is the discord between patient and physician perceptions of symptom burden, treatment goals, and expectations. New technologies, such as app-based reporting, can aid this communication, but are still not widely implemented. Additionally, regional variation further affects the psychosocial burden of MPNs on patients and their associates, as treatments and access to clinical trials are options for patients living in some areas, but not others. Overcoming some of the challenges in patient-physician communication and treatment access are key to improving disease management and QoL, as well as giving the patient greater input in treatment decisions.

Myeloproliferative neoplasms (MPNs) are a group of blood diseases where the body makes too many blood cells. Patients with MPNs can have symptoms which interfere with their daily lives, such as tiredness, pain, sweating at night, dizziness, itching, and difficulty sleeping. They also often suffer from anxiety and/or depression. In nearly all cases, physicians cannot cure the disease, but drugs can prevent blood clots and reduce the speed at which the disease gets worse. Usually, the main aim of treatment is improving patients' quality of life (QoL). Targeted drugs, such as ruxolitinib, treat MPNs and reduce symptoms, but do not cure the disease. Patients frequently want to play a bigger part in decisions about their treatment. However, physicians and patients often have different views on how well treatments are working and what to expect from the treatment. This can mean that patients feel they are not getting the best treatment for their symptoms. Also, patients may not be able to get some treatments or take part in a trial of a new drug, depending on where they live. This creates feelings of unfairness which can affect their mental health. Addressing all these problems may help improve the QoL for patients with these blood diseases.

Online yoga in myeloproliferative neoplasm patients: results of a randomized pilot trial to inform future research.

BACKGROUND: Myeloproliferative neoplasm (MPN) patients suffer from significant symptoms, inflammation and reduced quality of life. Yoga improves these outcomes in other cancers, but this hasn't been demonstrated in MPNs. The purpose of this study was to: (1) explore the limited efficacy (does the program show promise of success) of a 12-week online yoga intervention among MPN patients on symptom burden and quality of life and (2) determine feasibility (practicality: to what extent a measure can be carried out) of remotely collecting inflammatory biomarkers. METHODS: Patients were recruited nationally and randomized to online yoga (60 min/week of yoga) or wait-list control (asked to maintain normal activity). Weekly yoga minutes were collected with Clicky (online web analytics tool) and self-report. Those in online yoga completed a blood draw at baseline and week 12 to assess inflammation (interleukin-6, tumor necrosis factor-alpha [TNF-α]). All participants completed questionnaires assessing depression, anxiety, fatigue, pain, sleep disturbance, sexual function, total symptom burden, global health, and quality of life at baseline, week seven, 12, and 16. Change from baseline at each time point was computed by group and effect sizes were calculated. Pre-post intervention change in inflammation for the yoga group was compared by t-test. RESULTS: Sixty-two MPN patients enrolled and 48 completed the intervention (online yoga = 27; control group = 21). Yoga participation averaged 40.8 min/week via Clicky and 56.1 min/week via self-report. Small/moderate effect sizes were generated from the yoga intervention for sleep disturbance (d = - 0.26 to - 0.61), pain intensity (d = - 0.34 to - 0.51), anxiety (d = - 0.27 to - 0.37), and depression (d = - 0.53 to - 0.78). A total of 92.6 and 70.4% of online yoga participants completed the blood draw at baseline and week 12, respectively, and there was a decrease in TNF-α from baseline to week 12 (- 1.3 ± 1.5 pg/ml). CONCLUSIONS: Online yoga demonstrated small effects on sleep, pain, and anxiety as well as a moderate effect on depression. Remote blood draw procedures are feasible and the effect size of the intervention on TNF-α was large. Future fully powered randomized controlled trials are needed to test for efficacy. TRIAL REGISTRATION: This trial was retrospectively registered with clinicaltrials.gov (ID: NCT03503838 ) on 4/19/2018.

Perceptions of Myeloproliferative Neoplasm Patients Participating in an Online Yoga Intervention: A Qualitative Study.

Myeloproliferative neoplasms (MPNs) are rare hematological malignancies with a significant symptom burden often left unresolved despite recent advances in pharmacological therapy. Yoga is a nonpharmacological strategy that has been shown to improve symptoms in other cancers and may be effective for improving symptoms in MPN patients. Online yoga helps address many of the commonly reported barriers of cancer patients to in-person interventions and may make yoga more accessible to MPNs. An exploration of MPN patient perceptions of participation in online yoga is needed to tailor interventions to patient needs and inform future studies. The purpose of this study was to explore the perceptions of MPN patients participating in a 12-week online yoga intervention. This article represents the combined qualitative interview data from two studies. Participants were asked to complete 60 min/wk of online, home-based yoga and were asked to participate in a 15- to 20-minute phone interview postintervention. The qualitative data was coded in NVivo 11 for content analysis. The total sample included 39 MPN patients. Online yoga was well accepted and liked among these patients. They reported physical (eg, improved sleep, reduced fatigue) and mental (eg, reduced stress) health benefits and liked the convenience of being able to do yoga at home. Online yoga provides a feasible and attractive format through which to deliver a nonpharmacological intervention among MPN patients. Randomized controlled trials are needed to confirm the effects of online yoga on MPN patient symptoms. The qualitative findings presented here help inform the development of these future trials.

The effectiveness of exercise-based rehabilitation to patients with myeloproliferative neoplasms-An explorative study.

The aim of the study was to determine the impact of an interdisciplinary exercise-based rehabilitation intervention on fatigue and quality of life (QOL) in patients with Philadelphia-negative myeloproliferative neoplasms (MPNs). At the Danish Knowledge Centre for Rehabilitation and Palliative Care, a 5-day interdisciplinary exercise-based rehabilitation intervention was carried out on 48 patients with MPN. It was followed by 12 weeks of self-exercising prior to follow-up. Initially and at follow-up, participants filled out validated questionnaires; Brief Fatigue Inventory, Multidimensional Fatigue Inventory, European Organization for Research and Treatment of Cancer Quality-of-Life questionnaire, Myeloproliferative Neoplasm Symptom Assessment Form and Hospital Anxiety and Depression Scale. Maximal oxygen uptake and muscular strength were tested as well. Paired t test was used to compare scores between baseline and follow-up. In total, 45 participants (94%) completed the follow-up. No significant differences were observed on fatigue or QOL when comparing baseline and follow-up. Mean maximal oxygen uptake increased from 27.2 to 33.6 ml O2 · kg-1  ·min-1 (p < 0.001). Handgrip strength (p = 0.01) and the 30-s chair-stand test (p < 0.001) improved as well. No changes were found regarding levels of fatigue and QOL. However, we observed a significant increase in the physical capacity. Our observations call for further studies investigating the effects of non-pharmacological approaches in patients with MPN.

Social Media and Internet Resources for Patients with Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN).

The incorporation of Internet resources and the use of social media among patients, clinicians, advocates, and researchers in the field of hematology and oncology are growing in importance. Utilization of online information sharing is rising, especially among those involved in rare blood cancer fields, which have generally featured a paucity of reliable, updated information. In particular, blastic plasmacytoid dendritic cell neoplasm (BPDCN), an uncommon, but highly aggressive hematologic malignancy, is one example of a cancer with limited information readily available to the general public. The infrequent incidence of BPDCN, the challenges in recognizing the disease and making a clinico-pathologic diagnosis, and the lack of standard therapies are some of the reasons accounting for the dearth of expert opinion, scientific publications and discussion, and accessibility of online information for patients. This article highlights social media and Internet sources available for patients and other healthcare stakeholders in the field of BPDCN and discusses our efforts to increase awareness and propagation of BPDCN electronic resources, including the founding of an online Twitter community, #BPDCN.

Social Media and Myeloproliferative Neoplasms (MPN): Analysis of Advanced Metrics From the First Year of a New Twitter Community: #MPNSM.

The social media platform Twitter has provided the hematology/oncology community with unprecedented, novel methods of interpersonal communication and increased ability for the dissemination of important updates in a rapidly moving field. The advent, and subsequent success, of disease-specific Twitter communities have further enabled interested healthcare stakeholders to become quickly organized around a unique set of rare medical conditions, such as hematologic malignancies, that, historically, generally lack large amounts of reliable online information. One example is the Twitter community #MPNSM (myeloproliferative neoplasms on social media), which was started approximately one and half years ago and has served as a recognized venue for discussion among many members of the MPN community, including patients, researchers, providers, and advocacy organizations. This article will focus on understanding the impact of the founding of this community via the analysis of advanced Twitter metrics of user experience, from the first year of use for this novel healthcare hashtag.

The role of sexuality symptoms in myeloproliferative neoplasm symptom burden and quality of life: An analysis by the MPN QOL International Study Group.

BACKGROUND: Patients with myeloproliferative neoplasms (MPNs) including polycythemia vera, essential thrombocythemia, and myelofibrosis, are faced with oppressive symptom profiles that compromise daily functioning and quality of life. Among these symptoms, sexuality-related symptoms have emerged as particularly prominent and largely unaddressed. In the current study, the authors evaluated how sexuality symptoms from MPN relate to other patient characteristics, disease features, treatments, and symptoms. METHODS: A total of 1971 patients with MPN (827 with essential thrombocythemia, 682 with polycythemia vera, 456 with myelofibrosis, and 6 classified as other) were prospectively evaluated and patient responses to the Myeloproliferative Neoplasm Symptom Assessment Form (MPN-SAF) and the European Organization for Research and Treatment of Cancer Quality of Life Questionnaire Core 30 (EORTC-QLQ C30) were collected, along with information regarding individual disease characteristics and laboratory data. Sexuality scores were compared with an age-matched, healthy control population. RESULTS: Overall, patients with MPN were found to have greater sexual dysfunction compared with the healthy population (MPN-SAF score of 3.6 vs 2.0; P<.001), with 64% of patients with MPN describing some degree of sexual dysfunction and 43% experiencing severe symptoms. The presence of sexual symptoms correlated closely with all domains of patient functionality (physical, social, cognitive, emotional, and role functioning) and were associated with a reduced quality of life. Sexual problems also were found to be associated with other MPN symptoms, particularly depression and nocturnal and microvascular-related symptoms. Sexual dysfunction was more severe in patients aged >65 years and in those with cytopenias and transfusion requirements, and those receiving certain therapies such as immunomodulators or steroids. CONCLUSIONS: The results of the current study identify the topic of sexuality as a prominent issue for the MPN population, and this area would appear to benefit from additional investigation and management. Cancer 2016;122:1888-96. © 2016 American Cancer Society.

A new internet-based tool for reporting and analysing patient-reported outcomes and the feasibility of repeated data collection from patients with myeloproliferative neoplasms.

PURPOSE: An Internet-based tool for reporting and analysing patient-reported outcomes (PROs) has been developed. The tool enables merging PROs with blood test results and allows for computation of treatment responses. Data may be visualized by graphical analysis and may be exported for downstream statistical processing. The aim of this study was to investigate, whether patients with myeloproliferative neoplasms (MPNs) were willing and able to use the tool and fill out questionnaires regularly. METHODS: Participants were recruited from the outpatient clinic at the Department of Haematology, Roskilde University Hospital, Denmark. Validated questionnaires that were used were European Organisation for Research and Treatment of Cancer Quality of Life Questionnaire-Core 30, Myeloproliferative Neoplasm Symptom Assessment Form, Brief Fatigue Inventory and Short Form 36 Health Survey. Questionnaires were filled out ≥ 6 months online or on paper according to participant preference. Regularity of questionnaire submission was investigated, and participant acceptance was evaluated by focus-group interviews. RESULTS: Of 135 invited patients, 118 (87 %) accepted participation. One hundred and seven participants (91 %) preferred to use the Internet-based tool. Of the 118 enrolled participants, 104 (88 %) submitted PROs regularly ≥ 6 months. The focus-group interviews revealed that the Internet-based tool was well accepted. CONCLUSION: The Internet-based approach and regular collection of PROs are well accepted with a high participation rate, persistency and adherence in a population of MPN patients. The plasticity of the platform allows for adaptation to patients with other medical conditions.

Quality of life in MPN comes of age as a therapeutic target.

BCR-ABL-negative myeloproliferative neoplasms include primary myelofibrosis, polycythemia vera, and essential thrombocythemia. Clonal stem cell proliferation and dysregulated JAK/STAT molecular pathways characterize these hematologic malignancies. Symptoms experienced by patients are heterogeneous including excessive and disabling fatigue, early satiety, anorexia, pruritus, bone pain, night sweats, cachexia, abdominal pain and discomfort, and cognitive complaints. Patients also experience impaired quality of life along with decreased overall survival. New targeted drug therapies, including JAK2 inhibitors, have demonstrated remarkable success in alleviating the myeloproliferative neoplasm (MPN) symptomatic burden, reducing splenomegaly and improving quality of life while offering overall survival benefit. Within the USA, FDA approval has only been granted to use JAK2 inhibitors in intermediate- to high-risk myelofibrosis. However, given that low-prognostic-scoring patients have been shown to have considerable symptomology, there is a possibility that lower-risk patients may benefit from therapy. More than ever, the need for accurate MPN symptom burden assessment and subsequent addition of targeted therapies is critical in the treatment of MPNs. This article discusses the role of MPN symptom burden and quality of life as therapeutic targets in the context of recent MPN clinical advances.

Evaluating the utility of existing patient-reported outcome scales in novel patient populations with pancreatic cancer, lung cancer, and myeloproliferative neoplasms using medicare current beneficiary survey data.

BACKGROUND: While there are validated patient-reported outcomes (PRO) instruments for use in specific cancer populations, no validated general instruments exist for use in conditions common to multiple cancers, such as muscle wasting and consequent physical disability. The Medicare Current Beneficiary Survey (MCBS), a survey in a nationally representative sample of Medicare beneficiaries, includes items from three well known scales with general applicability to cancer patients: Katz activities of daily living (ADL), Rosow-Breslau instrumental ADL (IADL), and a subset of physical performance items from the Nagi scale. OBJECTIVE: This study evaluated properties of the Katz ADL, Rosow-Breslau IADL, and a subset of the Nagi scale in patients with pancreatic cancer, lung cancer, and myeloproliferative neoplasms (MPN) using data from MCBS linked with Medicare claims in order to understand the potential utility of the three scales in these populations; understanding patient-perceived significance was not in scope. METHODS: The study cohorts included Medicare beneficiaries aged ≥65 years as of 1 January of the year of their first cancer diagnosis with one or more health assessments in a community setting in the MCBS Access to Care data from 1991 to 2009. Beneficiaries had at least two diagnoses in de-identified Medicare claims data linked to the MCBS for one of the following cancers: pancreatic, lung, or MPN. The Katz ADL, Rosow-Breslau IADL, and Nagi scales were calculated to assess physical functioning over time from cancer diagnosis. Psychometric properties for each scale in each cohort were evaluated by testing for internal consistency, test-retest reliability, and responsiveness by comparing differences in mean scale scores over time as cancer progresses, and differences in mean scale scores before and after hospitalization (for lung cancer cohort). RESULTS: The study cohorts included 90 patients with pancreatic cancer, 863 with lung cancer, and 135 with MPN. Among each cancer cohort, the Katz ADL, Rosow-Breslau IADL, and Nagi scales had acceptable internal consistency (Cronbach's alpha generally between 0.70 and 0.90) and test-retest reliability for consecutive surveys before diagnosis and consecutive surveys after diagnosis (when patients' functioning was more stable). Compared with mean scale scores at the survey 1-2 years before cancer diagnosis (baseline), mean scale scores at the first survey after cancer diagnosis were significantly higher (P < 0.05), indicating worsening, for Katz ADL, Rosow-Breslau IADL, and Nagi scales (items scored 0-1) (0.54 vs. 1.45, 1.15 vs. 2.20, and 2.29 vs. 3.08, respectively, for pancreatic cancer; 0.73 vs. 1.24, 1.29 vs. 2.01, and 2.41 vs. 2.85 for lung cancer; and 0.44 vs. 0.86, 0.87 vs. 1.36, and 1.87 vs. 2.32 for MPN). Among lung cancer patients, scale scores increased significantly following a hospitalization, suggesting a worsening of functional status. CONCLUSIONS: The Katz ADL, Rosow-Breslau IADL, and Nagi scales collected in the MCBS demonstrate acceptable internal consistency and test-retest reliability among patients with pancreatic cancer, lung cancer, and MPN, and are consistent with clinical worsening following diagnosis or hospitalization. These results suggest that using retrospective data may allow researchers to conduct preliminary assessments of existing PRO instruments in new populations of interest and generate useful exploratory disease information before embarking on de novo PRO development.

Patients with polycythemia vera have worst impairment of quality of life among patients with newly diagnosed myeloproliferative neoplasms.

The quality of life (QoL) at the time of diagnosis of myeloproliferative neoplasm (MPN) has, to date, not been studied. One hundred and seventy-nine patients with MPN: 80 with essential thrombocythemia (ET), 73 with polycythemia vera (PV), 22 with primary myelofibrosis (PMF) and four with MPN undifferentiated, were included in this study. European Organisation for Research and Treatment of Cancer Quality of Life Questionnaire Core 30 (EORTC-QLQC30) and the MPN-Symptom Assessment Form (MPN-SAF) were used to evaluate QoL. Fatigue was the most reported symptom in these patients. Patients with PV reported significantly higher mean scores for inactivity, dizziness, cough, itching, depression and lower total QoL compared to patients with ET. Patients with PV had significantly more headache and itching compared to patients with PMF. When the newly diagnosed patients with MPN were compared with a cohort of patients with MPN with mean disease duration of 7.8 years, the differences were most striking for patients with PMF, with significantly more fatigue, abdominal discomfort, concentration problems, insomnia, fever, weight loss and lower overall QoL developed over time.

Association between quality of life and clinical parameters in patients with myeloproliferative neoplasms.

The Myeloproliferative Neoplasm Symptom Assessment Form (MPN-SAF) is a validated quality of life (QoL) instrument. In our Swedish cohort of 114 patients the symptomatic burden was found to be severe, with fatigue reported in 88% of the patients and reduced QoL in the majority of patients. Patients with primary myelofibrosis had the highest scores, low QoL, for most MPN-SAF items, compared to patients with polycythemia vera and essential thrombocythemia. Higher age showed significant associations with the BFI (Brief Fatigue Inventory) score, early satiety, concentration problems, dizziness, insomnia, cough and weight loss. Blood values, disease duration and myelosuppressive treatment did not significantly associate with any of the MPN-SAF items, with the exception of higher hemoglobin, which correlated with sad mood. Male patients with MPN scored significantly higher as regards sexual problems and weight loss compared to female patients. Overall, the MPN-SAF was found to be a valid instrument for assessing symptomatic burden among this population.