Valiação Da Carga Sintomática Em Doentes Com Neoplasias Mieloproliferativas Utilizando A Escala MPN-10 – Estudo De Coorte Da Pupulação Portuguesa
Palavras-chave:
Gestão da doença, MPN-10, Neoplasias mieloproliferativas, Qualidade de vida, SintomasResumo
Introdução: As Neoplasias mieloproliferativas (NMP), incluindo policitemia vera (PV), trombocitemia essencial (TE) e mielofibrose (MF), impactam significativamente na qualidade de vida (QV) dos pacientes. A avaliação da carga de sintomas e monitorização continua usando a escala MPN (Symptom Assessment Form) permite detectar alterações sintomáticas que podem ser sinais de progressão da doença e podem ser usados como um indicador da necessidade de reavaliar a evolução da doença e / oua abordagem terapêutica. Métodos: Estudo multicêntrico prospectivo de doentes portugueses com NMP, incluindo dados demográficos e caracterização clínica dos doentes e carga sintomática da doença com base na escala NMP-10. Resultados: Foram incluídos 324 pacientes, proporção de homens para mulheres de 0,7: 1, idade mediana de 71 anos, duração mediana da doença> 3 anos. A maioria dos pacientes apresentou TE (63%), 24% PV e 13% MF. Cerca de 70% estavam a ser tratados com hidroxiureia, 31% estavam sob recomendação de aspirina em baixas doses, 9% precisavam de flebotomia, 4% receberam ruxolitinibe e <2% com interferon. Os sintomas mais relatados incluem fadiga, inatividade, pruridoe problemas de concentração. A pontuação total dos sintomas dos pacientes com PV e TE melhorou significativamente desde o início até a última visita de acompanhamento. O Prurido teve uma melhora significativa em relação à linha de base para pacientes com PV, como fadiga, inatividade, problemas de concentração e suores noturnos em pacientes com TE. Conclusões: A aplicação sistemática do MPN-10 permitiu um conhecimento mais aprofundado dos pacientes e dos seus sintomas, o que, somado às mudanças analíticas, motivou reajustes terapêuticos que levaram a ganhos de QV. Uma análise futura desta coorte deve ser orientada para refinar esses resultados.Downloads
Referências
Abruzzese, E., Niscola, P., Trawinska, M. M., & de Fabritiis, P. (2018). Chronic myeloproliferative disorders: is quality-of-life the new goal? Current Medical Research and Opinion, 34(7), 1345–1347. https://doi.org/10.1080/03007995.2018.1473245.
Anderson, L. A., Duncombe, A. S., Hughes, M., Mills, M. E., Wilson, J. C., & McMullin, M. F. (2012). Environmental, lifestyle, and familial/ethnic factors associated with myeloproliferative neoplasms. American Journal of Hematology, 87(2), 175–182. https://doi.org/10.1002/ajh.22212.
Anderson, L. A., James, G., Duncombe, A. S., Mesa, R., Scherber, R., Dueck, A. C., de Vocht, F., Clarke, M., & Mcmullin, M. F. (2015). Myeloproliferative neoplasm patient symptom burden and quality of life: Evidence of significant impairment compared to controls. American Journal of Hematology, 90(10), 864–870. https://doi.org/10.1002/ajh.24098.
Arber, D. A., Orazi, A., Hasserjian, R., Thiele, J., Borowitz, M. J., Le Beau, M. M., Bloomfield, C. D., Cazzola, M., & Vardiman, J. W. (2016). The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood, 127(20), 2391–2405. https://doi.org/10.1182/blood-2016-03-643544.
Azevedo, Ana P., Silva, S. N., De Lima, J. P., Reichert, A., Lima, F., Júnior, E., & Rueff, J. (2017). DNA repair genes polymorphisms and genetic susceptibility to philadelphia-negative myeloproliferative neoplasms in a portuguese population: The role of base excision repair genes polymorphisms. Oncology Letters, 13(6), 4641–4650. https://doi.org/10.3892/ol.2017.6065.
Azevedo, Ana P., Silva, S. N., Reichert, A., Lima, F., Júnior, E., & Rueff, J. (2018). Effects of polymorphic DNA genes involved in BER and caspase pathways on the clinical outcome of myeloproliferative neoplasms under treatment with hydroxyurea. Molecular Medicine Reports, 18(6), 5243–5255. https://doi.org/10.3892/mmr.2018.9535.
Azevedo, Ana Paula, Silva, S. N., Reichert, A., Lima, F., Júnior, E., & Rueff, J. (2017). Prevalence of the janus kinase 2 V617F mutation in philadelphianegative myeloproliferative neoplasms in a portuguese population. Biomedical Reports, 7(4), 370–376. https://doi.org/10.3892/br.2017.977.
Barbui, T., Barosi, G., Birgegard, G., Cervantes, F., Finazzi, G., Griesshammer, M., Harrison, C., Hasselbalch, H. C., Hehlmann, R., Hoffman, R., Kiladjian, J. J., Kröger, N., Mesa, R., McMullin, M. F., Pardanani, A., Passamonti,
F., Vannucchi, A. M., Reiter, A., Silver, R. T., & Tefferi, A. ... (2011). Philadelphia-negative classical myeloproliferative neoplasms: critical concepts and management recommendations from European leukemiaNet. Journal of Clinical Oncology, 29(6), 761–770. https://doi.org/10.1200/JCO.2010.31.8436.
Brochmann, N., Flachs, E. M., Christensen, A. I., Andersen, C. L., Juel, K., Hasselbalch, H. C., & Zwisler, A. D. (2017). A nationwide population-based cross-sectional survey of health-related quality of life in patients with myeloproliferative neoplasms in Denmark (MPNhealthSurvey): Survey design and characteristics of respondents and nonrespondents. Clinical Epidemiology, 9, 141–150. https://doi.org/10.2147/CLEP.S117587.
Brochmann, N., Flachs, E. M., Christensen, A. I., Bak, M., Andersen, C. L., Juel, K., Hasselbalch, H. C., Zwisler, A. D., & Rottmann, N. (2019). Anxiety and depression in patients with philadelphia-negative myeloproliferative neoplasms: A nationwide population-based survey in Denmark. Clinical Epidemiology, 11, 23–33. https://doi.org/10.2147/CLEP.S162688.
Byun, J. M., Kim, Y. J., Youk, T., Yang, J. J., Yoo, J., & Park, T. S. (2017). Real world epidemiology of myeloproliferative neoplasms: a population based study in Korea 2004–2013. Annals of Hematology, 96(3), 373–381. https://doi.org/10.1007/s00277-016-2902-9.
Cervantes, F., Dupriez, B., Pereira, A., Passamonti, F., Reilly, J. T., Morra, E., Vannucchi, A. M., Mesa, R. A., Demory, J. L., Barosi, G., Rumi, E., & Tefferi, A. (2009). New prognostic scoring system for primary myelofibrosis based on a study of the International working group for myelofibrosis research and treatment. Blood, 113(13), 2895–2901. https://doi.org/10.1182/blood-2008-07-170449.
Deadmond, M. A., & Smith-Gagen, J. A. (2015). Changing incidence of myeloproliferative neoplasms: trends and subgroup risk profiles in the USA, 1973–2011. Journal of Cancer Research and Clinical Oncology, 141(12), 2131–2138. https://doi.org/10.1007/s00432-015-1983-5.
Duncombe, A. S., Anderson, L. A., James, G., De Vocht, F., Fritschi, L., Mesa, R., Clarke, M., & McMullin, M. F. (2020). Modifiable lifestyle and medical risk factors associated with myeloproliferative neoplasms. HemaSphere, 4(1), 2–7. https://doi.org/10.1097/HS9.0000000000000327.
Emanuel, R. M., Dueck, A. C., Geyer, H. L., Kiladjian, J. J., Slot, S., Zweegman, S., Te Boekhorst, P. A. W., Commandeur, S., Schouten, H. C., Sackmann, F., Fuentes, A. K., Hernández-Maraver, D., Pahl, H. L., Griesshammer, M., Stegelmann, F., Doehner, K., Lehmann, T., Bonatz, K., Reiter, A., & Mesa, R. A. ... (2012). Myeloproliferative neoplasm (MPN) symptom assessment form total symptom score: Prospective international assessment of an abbreviated symptom burden scoring system among patients with MPNs. Journal of Clinical Oncology, 30(33), 4098–4103. https://doi.org/10.1200/JCO.2012.42.3863.
Geyer, H. L., Kosiorek, H., Dueck, A. C., Scherber, R., Slot, S., Zweegman, S., te Boekhorst, P. A. W., Senyak, Z., Schouten, H. C., Sackmann, F., Fuentes, A. K., Hernández-Maraver, D., Pahl, H. L., Griesshammer, M., Stegelmann, F., Döhner, K., Lehmann, T., Bonatz, K., Reiter, A., & Mesa, R. A. ... (2017). Associations between gender, disease features and symptom burden in patients with myeloproliferative neoplasms: An analysis by the MPN QOL international working group. Haematologica, 102(1), 85–93. https://doi.org/10.3324/haematol.2016.149559.
Geyer, H. L., Scherber, R. M., Dueck, A. C., Kiladjian, J. J., Xiao, Z., Slot, S., Zweegman, S., Sackmann, F., Fuentes, A. K., Hernández-Maraver, D., Döhner, K., Harrison, C. N., Radia, D., Muxi, P., Besses, C., Cervantes, F., Johansson, P. L., Andreasson, B., Rambaldi, A., & Mesa, R. A. ... (2014). Distinct clustering of symptomatic burden among myeloproliferative neoplasm patients: Retrospective assessment in 1470 patients. Blood, 123(24), 3803–3810. https://doi.org/10.1182/blood-2013-09-527903.
Harrison, C. N., Koschmieder, S., Foltz, L., Guglielmelli, P., Flindt, T., Koehler, M., Mathias, J., Komatsu, N., Boothroyd, R. N., Spierer, A., Perez Ronco, J., Taylor-Stokes, G., Waller, J., & Mesa, R. A. (2017). The impact of myeloproliferative neoplasms (MPNs) on patient quality of life and productivity: results from the international MPN Landmark survey. Annals of Hematology, 96(10), 1653–1665. https://doi.org/10.1007/s00277-017-3082-y.
Heppner, J., Nguyen, L. T., Guo, M., Naugler, C., & Rashid-Kolvear, F. (2019). Incidence of myeloproliferative neoplasms in Calgary, Alberta, Canada. BMC Research Notes, 12(1), 1–5. https://doi.org/10.1186/s13104-019-4321-1.
Johansson, P., Mesa, R., Scherber, R., Abelsson, J., Samuelsson, J., Birgegård, G., & Andréasson, B. (2012). Association between quality of life and clinical parameters in patients with myeloproliferative neoplasms. Leukemia and Lymphoma, 53(3), 441–444. https://doi.org/10.3109/10428194.2011.619608.
Koschmieder, S. (2020). How I Manage Thrombotic / Thromboembolic complications in myeloproliferative neoplasms how i treat: Thrombotische / thromboembolische komplikationen bei patienten mit myeloproliferativen neoplasien. Hamostaseologie, 40, 47–53.
Kucine, N. (2020). Myeloproliferative Neoplasms in Children, Adolescents, and Young Adults. Current Hematologic Malignancy Reports, 15(2), 141–148. https://doi.org/10.1007/s11899-020-00571-8.
McFarland, D. C., Shaffer, K. M., Polizzi, H., Mascarenhas, J., Kremyanskaya, M., Holland, J., & Hoffman, R. (2018). Associations of physical and psychologic symptom burden in patients with philadelphia chromosomenegative myeloproliferative neoplasms. Psychosomatics, 59(5), 472–480. https://doi.org/10.1016/j.psym.2018.01.006.
McMullin, M. F., & Anderson, L. A. (2020). Aetiology of myeloproliferative neoplasms. Cancers, 12(7), 1–11. https://doi.org/10.3390/cancers12071810.
Mesa, R. A., Niblack, J., Wadleigh, M., Verstovsek, S., Camoriano, J., Barnes, S., Tan, A. D., Atherton, P. J., Sloan, J. A., & Tefferi, A. (2007). The burden of fatigue and quality of life in myeloproliferative disorders (MPDs): An international internet-based survey of 1179 MPD patients. Cancer, 109(1), 68–76. https://doi.org/10.1002/cncr.22365.
Mesa, R., Boccia, R. V., Grunwald, M. R., Oh, S. T., Colucci, P., Paranagama, D., Parasuraman, S., & Stein, B. L. (2018). Patient-reported outcomes data from reveal at the time of enrollment (baseline): A prospective observational study of patients with polycythemia vera in the United States. Clinical Lymphoma, Myeloma and Leukemia, 18(9), 590–596. https://doi.org/10.1016/j.clml.2018.05.020.
Mesa, R., Mayo Clinic Cancer Center, C., Jamieson, C., Deininger, M. W., Hopkins Jason Gotlib, J., Klisovic, R. B., Oh, S., Podoltsev, N., Pollyea, D. A.,
Þ, M., Rampal, R., M Rein, L. A., Scott, B., Snyder, D. S., Stein, B. L., Þ Robert, M. H., Verstovsek, S., Wadleigh, M., Wang, E. S., & Sundar, H. ... (2016). NCCN Myeloproliferative Neoplasms Version 2.2017. NCCN Guidelines, 14(12), 1572–1611.
Mesa, R., Miller, C. B., Thyne, M., Mangan, J., Goldberger, S., Fazal, S., Ma, X., Wilson, W., Paranagama, D. C., Dubinski, D. G., Boyle, J., & Mascarenhas, J. O. (2016). Myeloproliferative neoplasms (MPNs) have a significant impact on patients’ overall health and productivity: The MPN Landmark survey. BMC Cancer, 16(1), 1–10. https://doi.org/10.1186/s12885-016-2208-2.
Moulard, O., Mehta, J., Fryzek, J., Olivares, R., Iqbal, U., & Mesa, R. A. (2014). Epidemiology of myelofibrosis, essential thrombocythemia, and polycythemia vera in the European Union. European Journal of Haematology, 92(4), 289–297. https://doi.org/10.1111/ejh.12256.
Passamonti, F., Cervantes, F., Vannucchi, A. M., Morra, E., Rumi, E., Pereira, A., Guglielmelli, P., Pungolino, E., Caramella, M., Maffioli, M., Pascutto, C., Lazzarino, M., Cazzola, M., & Tefferi, A. (2010). A dynamic prognostic model to predict survival in primary myelofibrosis: A study by the IWG-MRT (International Working Group for Myeloproliferative Neoplasms Research and Treatment). Blood, 115(9), 1703–1708. https://doi.org/10.1182/blood-2009-09-245837.
Petruk, C., & Mathias, J. (2020). The Myeloproliferative Neoplasm Landscape: A Patient’s Eye View. Advances in Therapy, 37(5), 2050–2070. https://doi.org/10.1007/s12325-020-01314-0.
Roaldsnes, C., Holst, R., Frederiksen, H., & Ghanima, W. (2017). Myeloproliferative neoplasms: trends in incidence, prevalence and survival in Norway. European Journal of Haematology, 98(1), 85–93. https://doi.org/10.1111/ejh.12788.
Rocha, A., Valério, E., Ricou, C., Sanchez, J., Freitas, J., Caeiro, M., Sarmento, M., Duarte, M., Ponte, S., & Frias, S. (2018). Guia de apoio para avaliação de sintomalogia em doentes com síndromes mieloproliferativos através da aplicação da escala mpn-10. Onco.News, 11(36), 41–44. https://doi. org/10.31877/on.2018.36.01.
Sánchez, J., Ricou, C., & Ponte, S. (2019). Papel da equipa de enfermagem na consulta multidisciplinar a doentes com neoplasias mieloproliferativas. Onco.News, 38, 24–28. https://www.onco.news/papel-da-equipa-de-enfermagem-na-consulta-multidisciplinar-a-doentes-com-neoplasias-mieloproliferativas/.
Scherber, R., Dueck, A. C., Johansson, P., Barbui, T., Barosi, G., Vannucchi, A. M., Passamonti, F., Andreasson, B., Ferarri, M. L., Rambaldi, A., Samuelsson, J., Birgegard, G., Tefferi, A., Harrison, C. N., Radia, D., & Mesa, R. A. (2011). The Myeloproliferative Neoplasm Symptom Assessment Form (MPN-SAF): International prospective validation and reliability trial in 402 patients. Blood, 118(2), 401–408. https://doi.org/10.1182/blood-2011-01-328955.
Scherber, R. M., Kosiorek, H. E., Senyak, Z., Dueck, A. C., Clark, M. M., Boxer, M. A., Geyer, H. L., McCallister, A., Cotter, M., Van Husen, B., Harrison, C. N., & Mesa, R. A. (2016). Comprehensively understanding fatigue in patients with myeloproliferative neoplasms. Cancer, 122(3), 477–485. https://doi.org/10.1002/cncr.29753.
Seguro, F. S., Teixeira, L. L. C., da Rosa, L. I., da Silva, W. F., Nardinelli, L., Bendit, I., & Rocha, V. (2020). Risk factors and incidence of thrombosis in a Brazilian cohort of patients with Philadelphia-negative myeloproliferative neoplasms. Journal of Thrombosis and Thrombolysis, 49(4), 667–672. https://doi.org/10.1007/s11239-019-02029-y.
Shallis, R. M., Wang, R., Davidoff, A., Ma, X., Podoltsev, N. A., & Zeidan, A. M. (2020). Epidemiology of the classical myeloproliferative neoplasms: The four corners of an expansive and complex map. Blood Reviews, 42, 100706. https://doi.org/10.1016/j.blre.2020.100706.
Snyder, R. A., & Chang, G. J. (2019). Financial toxicity: A growing burden for cancer patients. American College of Surgeons, 2014, 10–11. https://bulletin.facs.org/2019/09/financial-toxicity-a-growing-burden-for-cancer-patients/.
Tefferi, A., & Vardiman, J. W. (2008). Classification and diagnosis of myeloproliferative neoplasms: The 2008 World Health Organization criteria and point-of-care diagnostic algorithms. Leukemia, 22(1), 14–22. https://doi.org/10.1038/sj.leu.2404955.
Tefferi, Ayalew. (2012). Myeloproliferative neoplasms 2012: The John M. Bennett 80th birthday anniversary lecture. Leukemia Research, 36(12), 1481–1489. https://doi.org/10.1016/j.leukres.2012.08.011.
Tefferi, Ayalew, Guglielmelli, P., Larson, D. R., Finke, C., Wassie, E. A., Pieri, L., Gangat, N., Fjerza, R., Belachew, A. A., Lasho, T. L., Ketterling, R. P., Hanson, C. A., Rambaldi, A., Finazzi, G., Thiele, J., Barbui, T., Pardanani, A., & Vannucchi, A. M. (2014). Long-term survival and blast transformation in molecularly annotated essential thrombocythemia, polycythemia vera, and myelofibrosis. Blood, 124(16), 2507–2513. https://doi.org/10.1182/blood-2014-05-579136.
Tefferi, Ayalew, & Vainchenker, W. (2011). Myeloproliferative neoplasms: Molecular pathophysiology, essential clinical understanding, and treatment strategies. Journal of Clinical Oncology, 29(5), 573–582. https://doi.org/10.1200/JCO.2010.29.8711.
Titmarsh, G. J., Duncombe, A. S., Mcmullin, M. F., O’Rorke, M., Mesa, R., De Vocht, F., Horan, S., Fritschi, L., Clarke, M., & Anderson, L. A. (2014). How common are myeloproliferative neoplasms? A systematic review and metaanalysis. American Journal of Hematology, 89(6), 581–587. https://doi.org/10.1002/ajh.23690.
Vannucchi, A. M., & Guglielmelli, P. (2010). Advances in understanding and management of polycythemia vera. Current Opinion in Oncology, 22(6), 636–641. https://doi.org/10.1097/CCO.0b013e32833ed81c.
Vannucchi, A. M., & Guglielmelli, P. (2017). What are the current treatment approaches for patients with polycythemia vera and essential thrombocythemia? Hematology, 2017(1), 480–488. https://doi.org/10.1182/asheducation-2017.1.480.
Verstovsek, S. (2010). Therapeutic potential of Janus-activated kinase-2 inhibitors for the management of myelofibrosis. Clinical Cancer Research, 16(7), 1988–1996. https://doi.org/10.1158/1078-0432.CCR-09-2836.
Yu, J., Paranagama, D., Geyer, H. L., Parasuraman, S., & Mesa, R. (2019). Relationship between symptom burden and disability leave among patients with myeloproliferative neoplasms (MPNs): findings from the Living with MPN patient survey. Annals of Hematology, 98(5), 1119–1125. https://doi.org/10.1007/s00277-019-03610-4.
Yu, J., Parasuraman, S., Paranagama, D., Bai, A., Naim, A., Dubinski, D., & Mesa, R. (2018). Impact of Myeloproliferative neoplasms on patients’ employment status and work productivity in the United States: Results from the living with MPNs survey. BMC Cancer, 18(1), 1–9. https://doi.org/10.1186/s12885-018-4322-9.
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