Myeloproliferative neoplasms (MPNs) – Part 1: An overview of the diagnosis and treatment of the “classical” MPNs

Sabrina Fowlkes, Cindy Murray, Adrienne Fulford, Tammy De Gelder, Nancy Siddiq


Myeloproliferative neoplasms (MPNs) are rare, yet potentially life-threatening, disorders caused by overproliferation of bone marrow stem cells. The symptom burden experienced by patients with the BCR-ABL1-negative MPNs (also referred to as the classical MPNs, i.e., essential thrombocythemia [ET], polycythemia vera [PV] and myelofibrosis [MF]) can be significant and can negatively impact quality of life (QOL). Since patients with these MPNs can live for several years, thereby requiring long-term treatment and follow-up, nurses play an essential role in communicating with these patients, assessing their symptoms, and educating them on treatments and self-management strategies that can reduce their symptom burden. This article, which is the first of a two-part series, was developed to provide nurses and other healthcare professionals with a review of the diagnosis and treatment of the most common classical MPNs. The second article in this series (also available in this issue) will provide nurses with practical guidance for managing the symptom burden associated with MPNs in order to help enhance the overall health and well-being of patients living with these disorders.

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Abdel-Wahab, O.I., & Levine, R.L. (2009). Primary myelofibrosis: Update on definition, pathogenesis, and treatment. Annual Review of Medicine, 60, 233–45.

Arber, D.A., Orazi, A., Hasserjian, R., Thiele, J., Borowitz, M.J., Le Beau, M.M., Bloomfield, … Vardiman, J.W. (2016). The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood, 127(20), 2391–2405.

Begna, K., Abdelatif, A., Schwager, S., Hanson, C., Pardanani, A., & Tefferi A. (2016). Busulfan for the treatment of myeloproliferative neoplasms: The Mayo Clinic experience. Blood Cancer Journal, 6, e427.

Birgegård, G. (2016). The use of anagrelide in myeloproliferative neoplasms, with focus on essential thrombocythemia. Current Hematologic Malignancy Reports, 11(5), 348–355.

Cervantes, F., Dupriez, B., Pereira, A., Passamonti, F., Reilly, J.T., Morra, E., Vannucchi, A.M., … 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.

Finazzi, G., & Barbui, T. (2007). How I treat patients with polycythemia vera. Blood, 109(12), 5104–11.

Gangat, N., Caramazza, D., Vaidya, R., George, G., Begna, K., Schwager, S., Van Dyke, D., … Tefferi, A. (2011). DIPSS plus: A refined Dynamic International Prognostic Scoring System for primary myelofibrosis that incorporates prognostic information from karyotype, platelet count, and transfusion status. Journal of Clinical Oncology, 29(4), 392–397.

Geyer, H.L., & Mesa, R.A. (2014). Therapy for myeloproliferative neoplasms: When, which agent, and how? Blood, 124(24), 3529–37.

Harrison, C., Kiladjian, J.J., Al-Ali, H.K., Gisslinger, H., Waltzman, R., Stalbovskaya, V., McQuitty, M., … Barosi, G. (2012). JAK inhibition with ruxolitinib versus best available therapy for myelofibrosis. New England Journal of Medicine, 366(9), 787–98.

Harrison, C.N., Vannucchi, A.M., Kiladjian, J.J., Al-Ali, H.K., Gisslinger, H., Knoops, L., Cervantes, F., … Barbui, T. (2016). Long-term findings from COMFORT-II, a phase 3 study of ruxolitinib versus best available therapy for myelofibrosis. Leukemia, 30, 1701–1707.

Mesa, R.A., & Cortes, J. (2013). Optimizing the management of ruxolitinib in patients with myelofibrosis: the need for individualized dosing. Journal of Hematology & Oncology, 6, 79.

Mesa, R., Jamieson, C., Bhatia, R., Deininger, M.W., Gerds, A.T., Gojo, I., Gotlib, J., … Sundar, H. (2016). Myeloproliferative Neoplasms, Version 2.2017, NCCN Clinical Practice Guidelines in Oncology. Journal of the National Comprehensive Cancer Network, 14(12), 1572–1611.

Nangalia, J., & Green, T.R. (2014) The evolving genomic landscape of myeloproliferative neoplasms. Hematology. American Society of Hematology. Education Program, 2014, 287–296.

Passamonti, F., Cervantes, F, Vannucchi, A.M., Morra, E., Rumi, E., Pereira, A., Guglielmelli, P., … 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.

Passamonti, F., Maffioli, M., Caramazza, D., & Cazzola M. (2011). Myeloproliferative neoplasms: from JAK2 mutations discovery to JAK2 inhibitor therapies. Oncotarget, 2(6), 485–490.

Passamonti, F., Vannucchi, A.M., Cervantes, F., Harrison, C., Morra, E., Kantarjian, H., & Verstovsek, S. (2015). Ruxolitinib and survival improvement in patients with myelofibrosis. Leukemia, 29(3), 739–40.

Radaelli, F., Onida, F., Rossi, F.G., Zilioli, V.R., Colombi, M., Usardi, P., Calori, R., & Zanella, A. (2008). Second malignancies in essential thrombocythemia (ET): A retrospective analysis of 331 patients with long-term follow-up from a single institution. Hematology, 13(4), 195–202.

Rollison, D.E., Howlader, N., Smith, M.T., Strom, S.S., Merritt, W.D., Ries, L.A., Edwards, B.K., & List, A.F. (2008). Epidemiology of myelodysplastic syndromes and chronic myeloproliferative disorders in the United States, 2001-2004, using data from the NAACCR and SEER programs. Blood, 112(1), 45–52.

Ruxolitinib (Jakavi) Product Monograph. Novartis Pharmaceuticals Canada, Inc. March 2, 2017.

Sirhan, S., Busque, L., Foltz, L., Grewal, K., Hamm, C., Laferriere, N., Laneuville, P., … Gupta, V. (2015). Evolving therapeutic options for polycythemia vera: Perspectives of the Canadian Myeloproliferative Neoplasms Group. Clinical Lymphoma, Myeloma & Leukemia, 15, 715–27.

Tefferi, A., & Barbui, T. (2015). Polycythemia vera and essential thrombocythemia: 2015 update on diagnosis, risk-stratification and management. American Journal of Hematology, 90(2), 162–73.

Tefferi, A., & Barbui, T. (2017). Polycythemia vera and essential thrombocythemia: 2017 update on diagnosis, risk-stratification, and management. American Journal of Hematology, 92(1), 94–108.

Tefferi, A., & Pardanani, A. (2015). Myeloproliferative neoplasms: a contemporary review. JAMA Oncology, 1(1), 97–105.

Tefferi, A., Rumi, E., Finazzi, G., Gisslinger, H., Vannucchi, A.M., Rodeghiero, F., Randi, M.L., … Barbui, T. (2013). Survival and prognosis among 1,545 patients with contemporary polycythemia vera: an international study. Leukemia, 27(9), 1874–1881.

Tonkin, J., Francis, Y., Pattinson, A., Peters, T., Taylor, M., Thompson, R., & Wallis, L. (2012). Myeloproliferative neoplasms: Diagnosis, management and treatment. Nursing Standard, 26(51), 44–51.

Vannucchi, A.M., Kiladjian, J,J., Griesshammer, M., Masszi, T., Durrant, S., Passamonti, F., Harrison, C.N., … Verstovsek, S. (2015). Ruxolitinib versus standard therapy for the treatment of polycythemia vera. New England Journal of Medicine, 372(5), 426–435.

Verstovsek, S., Mesa, R.A., Gotlib, J., Levy, R.S., Gupta, V., DiPersio, J.F., Catalano, J.V., … Kantarjian, H.M. (2012). A double-blind, placebo-controlled trial of ruxolitinib for myelofibrosis. New England Journal of Medicine, 366(9), 799–807.

Verstovsek, S., Vannucchi, A.M., Griesshammer, M., Masszi, T., Durrant, S., Passamonti, F., Harrison, C.N., … Kiladjian, J.J. (2016). Ruxolitinib versus best available therapy in patients with polycythemia vera: 80-week follow-up from the RESPONSE trial. Haematologica, 101(7), 821–9.

Wolanskyj, A.P., Schwager, S.M., McClure, R.F., Larson, D.R., & Tefferi, A. (2006). Essential thrombocythemia beyond the first decade: life expectancy, long-term complication rates, and prognostic factors. Mayo Clinic Proceedings, 81(2), 159–66.


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