Current Quality-of-Life Aspects in Patients with Classical Ph-Negative Myeloproliferative Neoplasms in the Russian Federation: Results and Discussion of the National Observational Program MPN-QoL-2020

AUTOIMMUNE THROMBOCYTOPENIA , , ,

TI Ionova1,2,3,*, EA Andreevskaya4,*, EN Babich5,*, NB Bulieva6,7,*, OYu Vinogradova8,9,10,*, EM Volodicheva11,*, SV Voloshin12,13,14,*, NN Glonina15,*, SK Dubov16,*, NB Esef’eva17,*, AYu Zaritskey18,*, EE Zinina19,*, MO Ivanova20,*, TYu Klitochenko21,*, AV Kopylova22,*, AD Kulagin23,*, GB Kuchma24,25,*, OYu Li26,*, EG Lomaia18,*, AL Melikyan27,*, VYa Melnichenko3,*, SN Menshakova28,*, NV Minaeva29,*, TA Mitina30,*, EV Morozova23,*,TP Nikitina1,2,*, OE Ochirova31,*, AS Polyakov13,*, TI Pospelova32,*, AV Proidakov33,*, OA Rukavitsyn34,*, GSh Safuanova35,36,*, IN Subortseva27,*, MS Fominykh37,*, MV Frolova38,*, TV Shelekhova39,*, DG Sherstnev39,*, TV Shneider40,*, VA Shuvaev12,41,*, ZK Abdulkhalikova23,†, LV Anchukova38,†, IA Apanaskevich15,†, AN Arnautova22,†, MV Barabanshchikova23,†, NV Berlina34,†, AP Bityukov34,†, EA Gilyazitdinova27,†, VI Gilmanshina36,†, EK Egorova27,†, EV Efremova12,†, EB Zhalsanova31,†, EN Kabanova19,†, OB Kalashnikova20,†, AE Kersilova41,†, TI Kolosheinova27,†, PM Kondratovskii16,†, EV Koroleva28,†, AN Kotelnikova34,†, NA Lazareva16,†, NS Lazorko18,†, EV Lyyurova33,†, AS Lyamkina32,†, YuN Maslova20,†, ES Mileeva12,†, NE Mochkin3,†, EK Nekhai16,†, YaA Noskov13,†, ES Osipova29,†, MM Pankrashkina8,†, EV Potanina16,†, OD Rudenko25,†, TYu Rozhenkova36,†, EI Sbityakova18,†, NT Siordiya18,†, AV Talko16,†, EI Usacheva42,†, YuB Chernykh30,†, TV Chitanava18,†, KS Shashkina27,†, DI Shikhbabaeva8,†, KS Yurovskaya23,†

1 Saint Petersburg State University Hospital, 154 Fontanki nab., Saint Petersburg, Russian Federation, 198103

2 Multinational Center for Quality of Life Research, 1 Artilleriiskaya ul., Saint Petersburg, Russian Federation, 191014

3 NI Pirogov National Medical and Surgical Center, 70 Nizhnyaya Pervomaiskaya ul., Moscow, Russian Federation, 105203

4 Krai Clinical Hospital No. 1, 7 Kokhanskogo ul., Chita, Russian Federation, 672038

5 Yugry District Clinical Hospital, 40 Kalinina ul., Khanty-Mansiisk, Russian Federation, 628011

6 I Kant Baltic Federal University, 14 Aleksandra Nevskogo ul., Kaliningrad, Russian Federation, 236041

7 Clinical Hospital of Kaliningrad Region, 74 Klinicheskaya ul., Kaliningrad, Russian Federation, 236016

8 Moscow Municipal Center for Hematology, SP Botkin City Clinical Hospital, 5 2-i Botkinskii pr-d, Moscow, Russian Federation, 125284

9 NI Pirogov Russian National Research Medical University, 1 Ostrovityanova ul., Moscow, Russian Federation, 117997

10 Dmitry Rogachev National Research Center of Pediatric Hematology, Oncology and Immunology, 1 Samory Mashela ul., Moscow, Russian Federation, 117997

11 Tula Regional Clinical Hospital, 1A korp. 1 Yablochkova ul., Tula, Russian Federation, 300053

12 Russian Research Institute of Hematology and Transfusiology, 16 2-ya Sovetskaya ul., Saint Petersburg, Russian Federation, 191024

13 SM Kirov Military Medical Academy, 6 Akademika Lebedeva ul., Saint Petersburg, Russian Federation, 194044

14 II Mechnikov North-Western State Medical University, 47 Piskarevskii pr-t, Saint Petersburg, Russian Federation, 195067

15 SI Sergeev Krai Clinical Hospital No. 1, 9 Krasnodarskaya ul., Khabarovsk, Russian Federation, 680009

16 Krai Center of Hematology, Krai Clinical Hospital No. 2, 55 Russkaya ul., Vladivostok, Russian Federation, 690105

17 Ulyanovsk Regional Clinical Hospital, 7 III Internatsionala ul., Ulyanovsk, Russian Federation, 432017

18 VA Almazov National Medical Research Center, 2 Akkuratova ul., Saint Petersburg, Russian Federation, 197341

19 Surgut District Clinical Hospital, 14 Energetikov ul., Surgut, Russian Federation, 628408

20 Clinical and Diagnostic Center, IP Pavlov First Saint Petersburg State Medical University, 6/8 L’va Tolstogo ul., Saint Petersburg, Russian Federation, 197022

21 Volgograd Regional Clinical Oncology Dispensary, 78 Zemlyachki ul., Volgograd, Russian Federation, 400138

22 Lipetsk Municipal Hospital No. 3 “Svobodnyi sokol”, 10 Ushinskogo ul., Lipetsk, Russian Federation, 398007

23 RM Gorbacheva Research Institute of Pediatric Oncology, Hematology and Transplantation, Pavlov University, 12 Rentgena ul., Saint Petersburg, Russian Federation, 197022

24 Orenburg State Medical University, 6 Sovetskaya ul., Orenburg, Russian Federation, 460000

25 Orenburg Regional Clinical Hospital, 23 Aksakova ul., Orenburg, Russian Federation, 460018

26 Sakhalin Regional Clinical Hospital, 430 Mira pr-t, Yuzhno-Sakhalinsk, Russian Federation, 693004

27 National Research Center for Hematology, 4 Novyi Zykovskii pr-d, Moscow, Russian Federation, 125167

28 Regional Clinical Hospital, 105 Peterburgskoe sh., Tver, Russian Federation, 170036

29 Kirov Research Institute of Hematology and Transfusiology, 72 Krasnoarmeiskaya ul., Kirov, Russian Federation, 610027

30 MF Vladimirskii Moscow Regional Research Clinical Institute, 61/2 Shchepkina ul., Moscow, Russian Federation, 129110

31 NA Semashko Republican Clinical Hospital, 12 Pavlova ul., Ulan-Ude, Russian Federation, 670031

32 Novosibirsk State Medical University, 52 Krasnyi pr-t, Novosibirsk, Russian Federation, 630091

33 Komi Republican Oncology Dispensary, 46 Nyuvchimskoe sh., Syktyvkar, Republic of Komi, Russian Federation, 167904

34 NN Burdenko Main Military Clinical Hospital, 3 Gospital’naya pl., Moscow, Russian Federation, 105229

35 Bashkir State Medical University, 3 Lenina ul., Ufa, Republic of Bashkortostan, Russian Federation, 450008

36 GG Kuvatov Republican Clinical Hospital, 132 Dostoevskogo ul., Ufa, Republic of Bashkortostan, Russian Federation, 450005

37 Multispecialty Clinic “Skandinaviya”, AVA-PETER, 55A Liteinyi pr-t, Saint Petersburg, Russian Federation, 191014

38 Vologda Regional Clinical Hospital, 17 Lechebnaya ul., Vologda, Russian Federation, 160002

39 VI Razumovskii Saratov State Medical University, 6/9 53rd Strelkovoi Divizii ul., Saratov, Russian Federation, 410028

40 Leningrad Regional Clinical Hospital, 45 korp. 2A Lunacharskogo pr-t, Saint Petersburg, Russian Federation, 194291

41 VV Veresaev Municipal Clinical Hospital, 10 Lobnenskaya ul., Moscow, Russian Federation, 127644

42 SM Clinic, 19 korp. 1 Udarnikov pr-t, Saint Petersburg, Russian Federation, 195279

* Coordinators and members of Expert Panel.

† Program participants.

For correspondence: Tatyana Pavlovna Nikitina, MD, PhD, 1 Artilleriiskaya ul., Saint Petersburg, Russian Federation, 191014; e-mail: qolife@mail.ru

For citation: Ionova TI, Andreevskaya EA, Babich EN, et al. Current Quality-of-Life Aspects in Patients with Classical Ph-Negative Myeloproliferative Neoplasms in the Russian Federation: Results and Discussion of the National Observational Program MPN-QoL-2020. Clinical oncohematology. 2022;15(2):176–97. (In Russ).

DOI: 10.21320/2500-2139-2022-15-2-176-197

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.

Keywords: classical Ph-negative myeloproliferative neoplasms, polycythemia vera, essential thrombocythemia, primary myelofibrosis, quality of life, MPN10 form.

Received: October 12, 2021

Accepted: February 10, 2022

Read in PDF

Статистика Plumx английский

REFERENCES

  1. Меликян А.Л., Ковригина А.М., Суборцева И.Н. и др. Национальные клинические рекомендации по диагностике и лечению Ph-негативных миелопролиферативных заболеваний (истинной полицитемии, эссенциальной тромбоцитемии, первичного миелофиброза) (редакция 2020 г.). Клиническая онкогематология. 2021;14(2):262–98. doi: 10.21320/2500-2139-2021-14-2-262-298.
    [Melikyan AL, Kovrigina AM, Subortseva IN, et al. National Clinical Guidelines on Diagnosis and Treatment of Ph-Negative Myeloproliferative Neoplasms (Polycythemia Vera, Essential Thrombocythemia, and Primary Myelofibrosis) (Edition 2020). Clinical oncohematology. 2021;14(2):262–98. doi: 10.21320/2500-2139-2021-14-2-262-298. (In Russ)]
  2. Geyer JT, Orazi A. Myeloproliferative neoplasms (BCR-ABL1 negative) and myelodysplastic/myeloproliferative neoplasms: current diagnostic principles and upcoming updates. Int J Lab Hematol. 2016;38(Suppl 1):12–9. doi: 10.1111/ijlh.12509.
  3. Mesa RA, Passamonti F. Individualizing Care for Patients With Myeloproliferative Neoplasms: Integrating Genetics, Evolving Therapies, and Patient-Specific Disease Burden. Am Soc Clin Oncol Educ. 2016;35:e324–e335. doi: 10.1200/EDBK_159322.
  4. Меликян А.Л., Суборцева И.Н., Шуваев В.А. и др. Современный взгляд на диагностику и лечение классических Ph-негативных миелопролиферативных заболеваний. Клиническая онкогематология. 2021;14(1):129–37. doi: 10.21320/2500-2139-2021-14-1-129-137.
    [Melikyan AL, Subortseva IN, Shuvaev VA, et al. Current View on Diagnosis and Treatment of Classical Ph-Negative Myeloproliferative Neoplasms. Clinical oncohematology. 2021;14(1):129–37. doi: 10.21320/2500-2139-2021-14-1-129-137. (In Russ)]
  5. Иванова М.О., Морозова Е.В., Барабанщикова М.В., Афанасьев Б.В. Ph-негативные миелопролиферативные новообра­зования: проблемы диагностики и терапии в России на примере Санкт-Петербурга. Клиническая онкогематология. 2021;14(1):45–52. doi: 10.21320/2500-2139-2021-14-1-45-52.
    [Ivanova MO, Morozova EV, Barabanshchikova MV, Afanasyev BV. Ph-Negative Myeloproliferative Neoplasms: Diagnosis and Treatment Challenges in Russia (the Case of Saint Petersburg). Clinical oncohematology. 2021;14(1):45–52. doi: 10.21320/2500-2139-2021-14-1-45-52. (In Russ)]
  6. Новик А.А., Ионова Т.И. Руководство по исследованию качества жизни в медицине. Под ред. Ю.А. Шевченко. 4-е изд., перераб. и доп. М.: Изд-во Национального медико-хирургического центра им. Н.И. Пирогова, 2021. 664 с.
    [Novik AA, Ionova TI. Rukovodstvo po issledovaniyu kachestva zhizni v meditsine. (Guide to the study of quality of life in medicine.) YuA Shevchenko, ed. 4th revised edition. Moscow: Natsional’nyi mediko-khirurgicheskii tsentr im. I. Pirogova Publ.; 2021. 664 p. (In Russ)]
  7. Mesa R, Miller CB, Thyne M, et al. Myeloproliferative neoplasms (MPNs) have a significant impact on patients’ overall health and productivity: the MPN Landmark survey. BMC Cancer. 2016;16:167. doi: 10.1186/s12885-016-2208-2.
  8. Mesa RA, Miller CB, Thyne M, et al. Differences in Treatment Goals and Perception of Symptom Burden Between Patients With Myeloproliferative Neoplasms (MPNs) and Hematologists/Oncologists in the United States: Findings From the MPN Landmark Survey. Cancer. 2017;123(3):449–58. doi: 10.1002/cncr.30325.
  9. Yu J, Parasuraman S, Paranagama D, et al. Impact of Myeloproliferative neoplasms on patients’ employment status and work productivity in the United States: results from the living with MPNs survey. BMC С 2018;18(1):420. doi: 10.1186/s12885-018-4322-9.
  10. Harrison CN, Koschmieder S, Foltz L, et al. The impact of myeloproliferative neoplasms (MPNs) on patient quality of life and productivity: results from the international MPN Landmark survey. Ann Hematol. 2017;96(10):1653–65. doi: 10.1007/s00277-017-3082-y.
  11. Xiao Z, Chang C-S, Morozova E, et al. Impact of myeloproliferative neoplasms (MPNs) and perceptions of treatment goals amongst physicians and patients in 6 countries: an expansion of the MPN Landmark Survey. HemaSphere. 2019;3(Suppl 1):294–5. doi: 10.1097/01.HS9.0000561008.75001.e7.
  12. Saydam G, Chang C, Morozova E, et al. Impact of myeloproliferative neoplasms (MPNs) and perceptions of treatment goals amongst physicians and patients in 6 countries: an expansion of the MPN Landmark Survey. Leuk Res. 2019;85:S60–S61. doi: 10.1016/S0145-2126(19)30353-4.
  13. Morozova EV, Barabanshchikova MV, Ionova TI, Afanasyev BV. Attitudes to the disease and therapy in patients with chronic Ph-negative myeloproliferative neoplasms: results of the physician and patient surveys in Russia as a part of International Landmark Study. Cell Ther Transplant. 2020;9(2):28–39. doi: 10.18620/ctt-1866-8836-2020-9-2-28-39.
  14. Качество жизни пациентов с миелопролиферативными новообразованиями и отношение пациентов и врачей к проблемам заболевания и лечения: результаты национальной наблюдательной программы МПН-КЖ-2020. Под ред. Т. И. Ионовой. М.: Практическая медицина, 2021. 36 с.
    [Ionova TI, ed. Kachestvo zhizni patsientov s mieloproliferativnymi novoobrazovaniyami i otnoshenie patsientov i vrachei k problemam zabolevaniya i lecheniya: rezul’taty natsional’noi nablyudatel’noi programmy MPN-KZh-2020. (Quality of life of patients with myeloproliferative neoplasms and patient- and doctor-reported attitudes to the disease and treatment issues: outcomes of the National Observational Program MPN-Qol-2020.) Moscow: Prakticheskaya meditsina Publ.; 2021. 36 p. (In Russ)]
  15. Меликян А.Л., Туркина А.Г., Абдулкадыров К.М. и др. Клинические рекомендации по диагностике и терапии Ph-негативных миелопролиферативных заболеваний (истинная полицитемия, эссенциальная тромбоцитемия, первичный миелофиброз). Гематология и трансфузиология. 2014;59(4):31–56.
    [Melikyan AL, Turkina AG, Abdulkadyrov KM, et al. Clinical guidelines for diagnosis and therapy of Ph-negative myeloproliferative neoplasms (polycythemia vera, essential thrombocythemia, primary myelofibrosis). Gematologiya i transfuziologiya. 2014;59(4):31–56. (In Russ)]
  16. Scherber R, Dueck AC, Johansson P, et al. The Myeloproliferative Neoplasm Symptom Assessment Form (MPN-SAF): international prospective validation and reliability trial in 402 patients. 2011;118(2):401–8. doi: 10.1182/blood-2011-01-328955.
  17. Emanuel RM, Dueck AC, Geyer HL, et al. Myeloproliferative Neoplasm (MPN) Symptom Assessment Form Total Symptom Score: Prospective International Assessment of an Abbreviated Symptom Burden Scoring System Among Patients with MPNs. J Clin Oncol. 2012;30(33):4098–103. doi: 10.1200/JCO.2012.42.3863.
  18. Ионова Т.И., Виноградова О.Ю., Ефремова Е.В. и др. Разработка и результаты апробации русской версии опросника MPN10 для оценки симптомов у пациентов с миелопролиферативными новообразованиями с учетом международных рекомендаций. Клиническая онкогематология. 2020;13(2):176–84. doi: 10.21320/2500-2139-2020-13-2-176-184.
    [Ionova TI, Vinogradova OYu, Efremova EV, et al. Development and Validation Results of the Russian MPN10 Form for Symptom Assessment in Patients with Myeloproliferative Neoplasms in Compliance with International Recommendations. Clinical oncohematology. 2020;13(2):176–84. doi: 10.21320/2500-2139-2020-13-2-176-184. (In Russ)]
  19. Goswami P, Oliva EN, Ionova TI, et al. Paper and electronic versions of HM-PRO, a novel patient-reported outcome measure for hematology: an equivalence study. J Comp Eff Res. 2019;8(7):523–33. doi: 10.2217/cer-2018-0108.
  20. Goswami P, Oliva EN, Ionova T, et al. Reliability of a novel haematological malignancy specific patient-reported outcome measure: HM-PRO. Front Pharmacol. 2020;11:571066. doi: 10.3389/fphar.2020.571066.
  21. Afanasyev B, Avtorhanova M, Bannikova M, et al. Implementation of Haematological Malignancies Patient Reported Outcome Measure in Clinical Practice: Haematologists’ Experience. Eur Med J Hematol. 2020;8(1):59–61.
  22. Petruk С, Mathias J. The Myeloproliferative Neoplasm Landscape: A Patient’s Eye View. Adv Ther. 2020;37(5):2050–70. doi: 10.1007/s12325-020-01314-0.
  23. Brochmann N, Flachs EM, Christensen AI, et al. Health-Related Quality of Life in Patients with Philadelphia-Negative Myeloproliferative Neoplasms: A Nationwide Population-Based Survey in Denmark. Cancers. 2020;12(12):3565. doi: 10.3390/cancers12123565.
  24. Gathany A, Scherber RM, Girardo M, et al. Myeloproliferative Neoplasm Quality of Life (MPN-QOL) Study Group: MPN Experimental Assessment of Symptoms By Utilizing Repetitive Evaluation (MEASURE) Trial. Blood. 2018;132(Suppl 1):1762. doi: 10.1182/blood-2018-99-111559.
  25. Langlais BT, Geyer H, Scherber R, et al. Quality of life and symptom burden among myeloproliferative neoplasm patients: do symptoms impact quality of life? Leuk Lymphoma. 2019;60(2):402–8. doi: 10.1080/10428194.2018.1480768.
  26. Mesa R, Palmer J, Eckert R, Huberty J. Quality of Life in Myeloproliferative Neoplasms: Symptoms and Management Implications. Hematol Oncol Clin North Am. 2021;35(2):375–90. doi: 10.1016/j.hoc.2020.12.006.