{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,4]],"date-time":"2026-09-04T18:07:00Z","timestamp":1788545220678,"version":"build-2803163510"},"reference-count":42,"publisher":"American Society of Hematology","issue":"1","content-domain":{"domain":["ashpublications.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2010,12,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>The seminal discovery of the JAK2V617F mutation, which is highly prevalent in Philadelphia-negative myeloproliferative disorders, now renamed neoplasms, triggered an almost unprecedented explosion of interest and data in the field. Descriptions of additional mutations in exon 12 of JAK2, at position 515 in MPL, and a number of other mutations at low frequency followed these discoveries. These advances in our understanding of molecular pathogenesis of these conditions coincided with the publication of results from two major clinical studies, ECLAP and PT-1, which contributed important clinical insights and facilitated significant correlative data collection. This article, focusing mainly upon essential thrombocythemia and polycythemia vera, reviews four major themes: the impact upon classification of these disorders considering a radical review of current terminology, and then three areas pertinent to clinical management: the indications for cytoreductive therapy in which the key targets are to reduce thrombohemorrhagic complications, relieve disease-related symptoms, and minimize the risk of transformation to secondary myeloid malignancy such as myelodysplasia, leukemia, and secondary myelofibrosis; and second reviewing current and, last, future therapeutic options, in particular interferon and JAK2 inhibitors.<\/jats:p>","DOI":"10.1182\/asheducation-2010.1.129","type":"journal-article","created":{"date-parts":[[2011,1,16]],"date-time":"2011-01-16T19:08:48Z","timestamp":1295204928000},"page":"129-134","update-policy":"https:\/\/doi.org\/10.1182\/blood.2019cm0000","source":"Crossref","is-referenced-by-count":16,"title":["Rethinking Disease Definitions and Therapeutic Strategies in Essential Thrombocythemia and Polycythemia Vera"],"prefix":"10.1182","volume":"2010","author":[{"given":"Claire","family":"Harrison","sequence":"first","affiliation":[{"name":"Guy's and St. Thomas' National Health Service Foundation Trust, London, United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"234","reference":[{"key":"2019111716593406000_B1","doi-asserted-by":"crossref","first-page":"372","DOI":"10.1182\/blood.V6.4.372.372","article-title":"Some speculations on the myeloproliferative syndromes","volume":"6","author":"Dameshek","year":"1951","journal-title":"Blood"},{"key":"2019111716593406000_B2","doi-asserted-by":"crossref","first-page":"2292","DOI":"10.1182\/blood-2002-04-1199","article-title":"The World Health Organization (WHO) classification of the myeloid neoplasms","volume":"100","author":"Vardiman","year":"2002","journal-title":"Blood"},{"key":"2019111716593406000_B3","doi-asserted-by":"crossref","first-page":"60","DOI":"10.1182\/blood-2007-05-091850","article-title":"Bone marrow pathology in essential thrombocythemia: interobserver reliability and utility for identifying disease subtypes","volume":"111","author":"Wilkins","year":"2008","journal-title":"Blood"},{"key":"2019111716593406000_B4","first-page":"1126","article-title":"Relevance of bone marrow features in the differential diagnosis between essential thrombocythemia and early stage idiopathic myelofibrosis","volume":"85","author":"Thiele","year":"2000","journal-title":"Haematologica"},{"key":"2019111716593406000_B5","doi-asserted-by":"crossref","first-page":"683","DOI":"10.1111\/j.1365-2141.2006.06348.x","article-title":"Concurrent MPL515 and JAK2V617F mutations in myelofibrosis: chronology of clonal emergence and changes in mutant allele burden over time","volume":"135","author":"Lasho","year":"2006","journal-title":"Br J Haematol"},{"key":"2019111716593406000_B6","doi-asserted-by":"crossref","first-page":"2003","DOI":"10.1182\/blood-2009-09-245381","article-title":"Clonal analysis of TET2 and JAK2 mutations suggests that TET2 can be a late event in the progression of myeloproliferative neoplasms","volume":"115","author":"Schaub","year":"2010","journal-title":"Blood"},{"key":"2019111716593406000_B7","doi-asserted-by":"crossref","first-page":"904","DOI":"10.1111\/j.1365-2141.2008.07560.x","article-title":"Clonal diversity in the myeloproliferative neoplasms: independent origins of genetically distinct clones","volume":"144","author":"Beer","year":"2009","journal-title":"Br J Haematol"},{"key":"2019111716593406000_B8","doi-asserted-by":"crossref","first-page":"201","DOI":"10.1182\/asheducation-2005.1.201","article-title":"Management of polycythemia vera and essential thrombocythemia","author":"Campbell","year":"2005","journal-title":"Hematology Am Soc Hematol Educ Program"},{"key":"2019111716593406000_B9","doi-asserted-by":"crossref","first-page":"1945","DOI":"10.1016\/S0140-6736(05)67785-9","article-title":"Definition of subtypes of essential thrombocythaemia and relation to polycythaemia vera based on JAK2 V617F mutation status: a prospective study","volume":"366","author":"Campbell","year":"2005","journal-title":"Lancet"},{"key":"2019111716593406000_B10","doi-asserted-by":"crossref","first-page":"1016","DOI":"10.1016\/j.exphem.2009.06.006","article-title":"JAK2V617F allele burden and thrombosis: a direct comparison in essential thrombocythemia and polycythemia vera","volume":"37","author":"Carobbio","year":"2009","journal-title":"Exp Hematol"},{"key":"2019111716593406000_B11","doi-asserted-by":"crossref","first-page":"67","DOI":"10.1016\/j.leukres.2008.06.006","article-title":"Is JAK2 V617F mutation more than a diagnostic index? 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