{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,13]],"date-time":"2026-07-13T13:53:24Z","timestamp":1783950804455,"version":"3.55.0"},"reference-count":41,"publisher":"American Society of Hematology","issue":"1","content-domain":{"domain":["ashpublications.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2009,1,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>The last four years have seen an explosion in our understanding of the myeloproliferative neoplasms. Important and often unexpected insights into the molecular mechanisms responsible for these disorders have been accompanied by the development of new diagnostic tests and by an improved understanding of the relationship between the different disease entities. This review will focus on recent developments in the pathogenesis and management of essential thrombocythemia with a particular emphasis on its phenotypic overlap with polycythemia vera and primary myelofibrosis.<\/jats:p>","DOI":"10.1182\/asheducation-2009.1.621","type":"journal-article","created":{"date-parts":[[2009,12,11]],"date-time":"2009-12-11T20:35:31Z","timestamp":1260563731000},"page":"621-628","update-policy":"https:\/\/doi.org\/10.1182\/blood.2019cm0000","source":"Crossref","is-referenced-by-count":30,"title":["Pathogenesis and management of essential thrombocythemia"],"prefix":"10.1182","volume":"2009","author":[{"given":"Philip A.","family":"Beer","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anthony R.","family":"Green","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"234","reference":[{"key":"2019111702484136100_R1","doi-asserted-by":"crossref","unstructured":"Campbell PJ, Green AR. The myeloproliferative disorders. N Engl J Med. 2006;355:2452\u20132466.","DOI":"10.1056\/NEJMra063728"},{"key":"2019111702484136100_R2","doi-asserted-by":"crossref","unstructured":"Levine RL, Pardanani A, Tefferi A, Gilliland DG. Role of JAK2 in the pathogenesis and therapy of myeloproliferative disorders. Nat Rev Cancer. 2007;7:673\u2013683.","DOI":"10.1038\/nrc2210"},{"key":"2019111702484136100_R3","doi-asserted-by":"crossref","unstructured":"Pikman Y, Lee BH, Mercher T, et al. MPLW515L is a novel somatic activating mutation in myelofibrosis with myeloid metaplasia. PLoS Med. 2006;3:e270.","DOI":"10.1371\/journal.pmed.0030270"},{"key":"2019111702484136100_R4","doi-asserted-by":"crossref","unstructured":"Beer PA, Campbell PJ, Scott LM, et al. MPL mutations in myeloproliferative disorders: analysis of the PT-1 cohort. 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N Engl J Med. 2007;356:459\u2013468.","DOI":"10.1056\/NEJMoa065202"},{"key":"2019111702484136100_R11","doi-asserted-by":"crossref","unstructured":"Lacout C, Pisani DF, Tulliez M, Gachelin FM, Vainchenker W, Villeval JL. JAK2V617F expression in murine hematopoietic cells leads to MPD mimicking human PV with secondary myelofibrosis. Blood. 2006;108:1652\u20131660.","DOI":"10.1182\/blood-2006-02-002030"},{"key":"2019111702484136100_R12","doi-asserted-by":"crossref","unstructured":"Tiedt R, Hao-Shen H, Sobas MA, et al. Ratio of mutant JAK2-V617F to wild-type Jak2 determines the MPD phenotypes in transgenic mice. Blood. 2008;111:3931\u20133940.","DOI":"10.1182\/blood-2007-08-107748"},{"key":"2019111702484136100_R13","doi-asserted-by":"crossref","unstructured":"Olthof SG, Fatrai S, Drayer AL, Tyl MR, Vellenga E, Schuringa JJ. Downregulation of signal transducer and activator of transcription 5 (STAT5) in CD34+ cells promotes megakaryocytic development, whereas activation of STAT5 drives erythropoiesis. Stem Cells. 2008;26:1732\u20131742.","DOI":"10.1634\/stemcells.2007-0899"},{"key":"2019111702484136100_R14","doi-asserted-by":"crossref","unstructured":"Campbell PJ, Bareford D, Erber WN, et al. Reticulin accumulation in essential thrombocythemia: prognostic significance and relationship to therapy. J Clin Oncol. 2009;27:2991\u20132999.","DOI":"10.1200\/JCO.2008.20.3174"},{"key":"2019111702484136100_R15","doi-asserted-by":"crossref","unstructured":"Bellanne-Chantelot C, Chaumarel I, Labopin M, et al. Genetic and clinical implications of the Val617Phe JAK2 mutation in 72 families with myeloproliferative disorders. Blood. 2006;108:346\u2013352.","DOI":"10.1182\/blood-2005-12-4852"},{"key":"2019111702484136100_R16","doi-asserted-by":"crossref","unstructured":"Landgren O, Goldin LR, Kristinsson SY, Helgadottir EA, Samuelsson J, Bjorkholm M. Increased risks of polycythemia vera, essential thrombocythemia, and myelofibrosis among 24,577 first-degree relatives of 11,039 patients with myeloproliferative neoplasms in Sweden. Blood. 2008;112:2199\u20132204.","DOI":"10.1182\/blood-2008-03-143602"},{"key":"2019111702484136100_R17","doi-asserted-by":"crossref","unstructured":"Olcaydu D, Harutyunyan A, Jager R, et al. A common JAK2 haplotype confers susceptibility to myeloproliferative neoplasms. Nat Genet. 2009;41:450\u2013454.","DOI":"10.1038\/ng.341"},{"key":"2019111702484136100_R18","doi-asserted-by":"crossref","unstructured":"Kilpivaara O, Mukherjee S, Schram AM, et al. A germline JAK2 SNP is associated with predisposition to the development of JAK2(V617F)-positive myeloproliferative neoplasms. 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