The main goals of therapy for MPNs are to prolong survival and improve the quality of life of patients.12
At this time, the only curative option for patients with MPNs is allogeneic stem cell transplant, but most patients are not candidates for this procedure.13 Most of the available therapies only treat individual clinical manifestations of MPNs without addressing the underlying disease.13
Although few medications have been globally approved for the treatment of MPNs, recent clinical trials have resulted in the approval of new modalities. Other clinical trials are still underway to find more therapeutic options in more indications.13-15
- 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.