title: Myeloproliferative neoplasm patient symptom burden and quality of life: evidence of significant impairment compared to controls.
abstract: The myeloproliferative neoplasms (MPN) including polycythaemia vera (PV), essential thrombocythaemia and primary myelofibrosis (PMF) are rare diseases contributing to significant morbidity. Symptom management is a prime treatment objective but current symptom assessment tools have not been validated compared to the general population. The MPN-symptom assessment form (MPN-SAF), a reliable and validated clinical tool to assess MPN symptom burden, was administered to MPN patients (n = 106) and, for the first time, population controls (n = 124) as part of a UK case-control study. Mean symptom scores were compared between patients and controls adjusting for potential confounders. Mean patient scores were compared to data collected by the Mayo Clinic, USA on 1,446 international MPN patients to determine patient group representativeness. MPN patients had significantly higher mean scores than controls for 25 of the 26 symptoms measured (P < 0.05); fatigue was the most common symptom (92.4% and 78.1%, respectively). Female MPN patients suffered worse symptom burden than male patients (P < 0.001) and substantially worse burden than female controls (P < 0.001). Compared to the Mayo clinic patients, MPN-UK patients reported similar symptom burden but lower satiety (P = 0.046). Patients with PMF reported the worst symptom burden (88.3%); significantly higher than PV patients (P < 0.001). For the first time we report quality of life was worse in MPN-UK patients compared with controls (P < 0.001).
authors.affiliation: Centre for Public Health, Queen's University Belfast, Belfast, Northern Ireland.
authors.forename: Lesley A
authors.initials: LA
authors.lastname: Anderson
authors.affiliation: Centre for Public Health, Queen's University Belfast, Belfast, Northern Ireland.
authors.forename: Glen
authors.initials: G
authors.lastname: James
authors.affiliation: Department of Haematology, University Hospitals Southampton NHS Foundation Trust, Hampshire, United Kingdom.
authors.forename: Andrew S
authors.initials: AS
authors.lastname: Duncombe
authors.affiliation: Mayo Clinic Cancer Centre, Scottsdale, Arizona.
authors.forename: Ruben
authors.initials: R
authors.lastname: Mesa
authors.affiliation: Mayo Clinic Cancer Centre, Scottsdale, Arizona.
authors.forename: Robyn
authors.initials: R
authors.lastname: Scherber
authors.affiliation: Mayo Clinic Cancer Centre, Scottsdale, Arizona.
authors.forename: Amylou C
authors.initials: AC
authors.lastname: Dueck
authors.affiliation: School of Social and Community Medicine, University of Bristol, Bristol, United Kingdom.
authors.forename: Frank
authors.initials: F
authors.lastname: de Vocht
authors.affiliation: Centre for Public Health, Queen's University Belfast, Belfast, Northern Ireland.
authors.forename: Mike
authors.initials: M
authors.lastname: Clarke
authors.affiliation: Centre for Cancer Research and Cell Biology, Queen's University Belfast, Belfast, Northern Ireland.
authors.forename: Mary F
authors.initials: MF
authors.lastname: McMullin
mesh_terms: D017281:Cost of Illness; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D009196:Myeloproliferative Disorders / Q000628:therapy*; D011788:Quality of Life