Keywords
polycythemia vera
essential thrombocythemia
primary myelofibrosis
quality of life
MPN10 form
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Keywords
Abstract
Background. The National Observational Program MPN-QoL-2020 was aimed at collecting the data on QoL (quality of life) characteristics and symptoms as well as patient- and physician-related disease and treatment perceptions in classical Ph-negative myeloproliferative neoplasms (MPN) in the Russian Federation.
Aim. Using new standardized forms, to analyze the quality of life among patients with various MPNs, to characterize ubiquitous symptoms and their effect on quality of life among the myelofibrosis (MF), polycythemia vera (PV), and essential thrombocythemia (ET) patients as well as to describe the perceptions of disease- and therapy-associated problems as reported by patients and hematologists treating them.
Materials & Methods. The study enrolled 1100 patients with Ph-negative MPNs (355 MF, 408 PV, and 337 ET patients at the mean age of 58 ± 14 years, 61 % women). The study also involved 100 hematologists (mean age of 42 ± 12 years, 85 % women) from 37 health and preventive facilities in 8 Federal districts of the Russian Federation. The patients contributed to the study by one-time completing a special MPN10 form for MPN symptom assessment, a special QoL questionnaire HM-PRO for hematological malignancy patients, as well as a patient checklist. The task of hematologists consisted in one-time filling out of a physician checklist and completing the medical records of all the enrolled MPN patients.
Results. For the first time in the Russian Federation, the real clinical practice yielded the data on the quality of life in Ph-negative MPN patients, symptom profiles in different MPNs, and the extent of their effect on everyday life. QoL impairments mostly relate to physical and emotional functioning of MPN patients and to feeding and drinking regime, but rarely to social functioning. More than 1/3 of patients with Ph-negative MPNs reported on considerable QoL impairments. Absolute majority of patients complain of weakness: 92.6 % in MF, 83.7 % in PV, and 82 % in ET. The profiles of relevant symptoms and their intensity differ in various MPNs. The study identified the symptoms which need most to be corrected, both in the view of patients and physicians. There were established differences between patient- and doctor-reported evaluations of the attitude to the disease and treatment as well as the aspects for improvement in physician-patient relationship.
Conclusion. The National Observational Program MPN-QoL-2020 has resulted in characterization of QoL impairments in MPN patients in Russia. It determined the spectrum of particular disease and treatment challenges specific to these patients. Moreover, their unmet needs were updated. The outcomes of MPN-QoL-2020 can serve as a basis for the guidelines for QoL improvement/maintenance in Ph-negative MPNs and for activities aimed at raising MPN patients’ awareness about the disease and its treatment.
References
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