MPNs are a group of clonal disorders that affect multipotent hematopoietic progenitor cells.1,2 They are rare but potentially life-threatening diseases that can severely affect the quality of life of patients due to debilitating symptoms and an increased risk of cardiovascular events.3
In patients with MPNs, the type of differentiated myeloid cell that is affected determines which MPN develops and can impact mortality.1
The classic Philadelphia chromosome–negative MPNs—MF, PV, and ET—are heterogeneous diseases with distinct clinical characteristics, but they share several features in their pathogenesis and symptomatology.2,4
Each year 0.5 to 3.2 new cases of MPNs are diagnosed in 100,000 people worldwide.5-7
- Swerdlow SH, Campo E, Harris NL, et al. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. 4th ed. Lyon, France: IARC Press; 2008.
- Spivak JL. Narrative review: thrombocytosis, polycythemia vera, and JAK2 mutations: the phenotypic mimicry of chronic myeloproliferation. Ann Intern Med. 2010;152(5):300-306.
- Cervantes F, Passamonti F, Barosi G. Life expectancy and prognostic factors in the classic BCR/ABL-negative myeloproliferative disorders. Leukemia. 2008;22(5):905-914.
- Delhommeau F, Jeziorowska D, Marzac C, Casadevall N. Molecular aspects of myeloproliferative neoplasms. Int J Hematol. 2010;91(2):165-173.
- Leukemia & Lymphoma Society. Disease information and support: myeloproliferative neoplasms, incidence. http://www.lls.org/#/diseaseinformation/myeloproliferativediseases/incidence/. Accessed October 6, 2014.
- Maynadié M, Girodon F, Manivet-Janoray I, et al. Twenty-five years of epidemiological recording on myeloid malignancies: data from the specialized registry of hematologic malignancies of Cote d'or (Burgundy, France). Haematologica. 2011;96(1):55-61.
- Visser O, Trama A, Maynadié M, et al. Incidence, survival and prevalence of myeloid malignancies in Europe. Eur J Cancer. 2012;48(17):3257-3266.
- Mesa RA, Niblack J, Wadleigh M, et al. The burden of fatigue and quality of life in myeloproliferative disorders (MPDs): an international Internet-based survey of 1179 MPD patients. Cancer. 2007;109(1):68-76.
- Smith CA, Fan G. The saga of JAK2 mutations and translocations in hematologic disorders: pathogenesis, diagnostic and therapeutic prospects, and revised World Health Organization diagnostic criteria for myeloproliferative neoplasms. Hum Pathol. 2008;39(6):795-810.
- Tefferi A. Novel mutations and their functional and clinical relevance in myeloproliferative neoplasms: JAK2, MPL, TET2, ASXL1, CBL, IDH and IKZF1. Leukemia. 2010;24(6):1128-1138.
- Nangalia J, Massie CE, Baxter EJ, et al. Somatic CALR mutations in myeloproliferative neoplasms with nonmutated JAK2. N Engl J Med. 2013;369(25):2391-2405.
- Barbui T, Barosi G, Birgegard G, et al. Philadelphia-negative classical myeloproliferative neoplasms: critical concepts and management recommendations from European LeukemiaNet. J Clin Oncol. 2011;29(6):761-770.
- Mesa RA. New drugs for the treatment of myelofibrosis. Curr Hematol Malig Rep. 2010;5(1):15-21.
- Beer PA, Green AR. Pathogenesis and management of essential thrombocythemia. Hematology Am Soc Hematol Educ Program. 2009:621-628.
- Leukemia & Lymphoma Society. Polycythemia vera facts. http://www.lls.org/content/nationalcontent/resourcecenter/freeeducationmaterials/mpd/pdf/polycythemiavera.pdf. Accessed October 6, 2014.