{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,28]],"date-time":"2026-03-28T22:55:56Z","timestamp":1774738556304,"version":"3.50.1"},"reference-count":36,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2017,5,16]],"date-time":"2017-05-16T00:00:00Z","timestamp":1494892800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100004963","name":"Seventh Framework Programme","doi-asserted-by":"publisher","award":["306242\u2010NGS\u2010PTL"],"award-info":[{"award-number":["306242\u2010NGS\u2010PTL"]}],"id":[{"id":"10.13039\/501100004963","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Hematological Oncology"],"published-print":{"date-parts":[[2018,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>An accurate histological diagnosis may distinguish essential thrombocythaemia (ET) from early primary myelofibrosis (early\u2010PMF), which is associated with worse outcome. Outcome of ET is also negatively affected by the presence of the <jats:italic>JAK2<\/jats:italic><jats:sup>V617F<\/jats:sup> mutation. To investigate the impact of <jats:italic>JAK2<\/jats:italic><jats:sup>V617F<\/jats:sup> mutation burden and histology on outcome, we collected 475 WHO\u2010diagnosed ET (69.2%) or early\u2010PMF <jats:italic>JAK2<\/jats:italic><jats:sup>V617F<\/jats:sup>\u2010positive patients followed in 4 Italian haematology centers. <jats:italic>JAK2<\/jats:italic><jats:sup>V617F<\/jats:sup> allele burden was \u226450% in 90% and 87% of ET and early\u2010PMF patients, respectively (<jats:italic>P<\/jats:italic>\u00a0=\u00a0.34). During follow\u2010up, 32 (9.7%) ET and 18 (12.3%) early\u2010PMF patients experienced 59 thrombotic events, and 27 patients (5.6%) and 6 (1.2%) patients evolved to myelofibrosis and acute leukemia, respectively. At last contact, 28 (5.8%) patients had died. In early\u2010PMF compared to ET, the 10\u2010year mortality rates (6.7% and 4.3%, <jats:italic>P<\/jats:italic>\u00a0=\u00a0.73), leukemic transformation rates (1.4% and 1.2%, <jats:italic>P<\/jats:italic>\u00a0=\u00a0.45), and thrombosis rates (16.7% and 12.2%, <jats:italic>P<\/jats:italic>\u00a0=\u00a0.12) were comparable. Only progression to overt myelofibrosis at 10\u00a0years was significantly worse (11.4% and 1.5%, <jats:italic>P<\/jats:italic>\u00a0=\u00a0.004). In multivariate analysis, a higher (&gt;50%) <jats:italic>JAK2<\/jats:italic><jats:sup>V617F<\/jats:sup> burden was significantly correlated with fibrotic progression and histology. Considering <jats:italic>JAK2<\/jats:italic><jats:sup>V617F<\/jats:sup>\u2010positive disease, a higher (&gt;50%) <jats:italic>JAK2<\/jats:italic><jats:sup>V617F<\/jats:sup> burden and histological classification are independent prognostic risk factors for disease progression. These findings reinforce the need for standardized detection of this mutation.<\/jats:p>","DOI":"10.1002\/hon.2430","type":"journal-article","created":{"date-parts":[[2017,5,16]],"date-time":"2017-05-16T10:03:07Z","timestamp":1494928987000},"page":"269-275","source":"Crossref","is-referenced-by-count":13,"title":["Comparison of <i>JAK2<\/i><sup>V617F<\/sup>\u2010positive essential thrombocythaemia and early primary myelofibrosis: The impact of mutation burden and histology"],"prefix":"10.1002","volume":"36","author":[{"given":"Roberto","family":"Latagliata","sequence":"first","affiliation":[{"name":"Division of Hematology\u2010Department of Cellular Biotechnologies and Hematology University La Sapienza of Rome  Rome Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nicola","family":"Polverelli","sequence":"additional","affiliation":[{"name":"Unit of Stem Cell Transplantation, Chair of Hematology ASST Spedali Civili di Brescia  Brescia Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alessia","family":"Tieghi","sequence":"additional","affiliation":[{"name":"Division of Hematology Azienda Ospedaliera\u2010IRCSS Arcispedale Santa Maria Nuova  Reggio Emilia Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Giuseppe Alberto","family":"Palumbo","sequence":"additional","affiliation":[{"name":"Division of Hematology, Ospedale Ferrarotto University of Catania  Catania Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Massimo","family":"Breccia","sequence":"additional","affiliation":[{"name":"Division of Hematology\u2010Department of Cellular Biotechnologies and Hematology University La Sapienza of Rome  Rome Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Elena","family":"Sabattini","sequence":"additional","affiliation":[{"name":"Haematopathology Unit, Department of Experimental, Diagnostic and Specialty Medicine, S. Orsola\u2010Malpighi Hospital University of Bologna  Bologna Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Loredana","family":"Villari","sequence":"additional","affiliation":[{"name":"Division of Hematopathology, Ospedale Ferrarotto University of Catania  Catania Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mara","family":"Riminucci","sequence":"additional","affiliation":[{"name":"Haematopathology Unit, Cellular Biotechnologies and Hematology University Sapienza  Rome Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Riccardo","family":"Valli","sequence":"additional","affiliation":[{"name":"Haematopathology Unit Azienda Ospedaliera Arcispedale Santa Maria Nuova  Reggio Emilia Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lucia","family":"Catani","sequence":"additional","affiliation":[{"name":"Department of Experimental, Diagnostic and Specialty Medicine University of Bologna  Bologna Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Giuliana","family":"Alimena","sequence":"additional","affiliation":[{"name":"Division of Hematology\u2010Department of Cellular Biotechnologies and Hematology University La Sapienza of Rome  Rome Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Emanuela","family":"Ottaviani","sequence":"additional","affiliation":[{"name":"Department of Experimental, Diagnostic and Specialty Medicine University of Bologna  Bologna Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Angelo","family":"Fama","sequence":"additional","affiliation":[{"name":"Division of Hematology Azienda Ospedaliera\u2010IRCSS Arcispedale Santa Maria Nuova  Reggio Emilia Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Giovanni","family":"Martinelli","sequence":"additional","affiliation":[{"name":"Department of Experimental, Diagnostic and Specialty Medicine University of Bologna  Bologna Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Margherita","family":"Perricone","sequence":"additional","affiliation":[{"name":"Department of Experimental, Diagnostic and Specialty Medicine University of Bologna  Bologna Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5098-3901","authenticated-orcid":false,"given":"Marco","family":"Spinsanti","sequence":"additional","affiliation":[{"name":"Department of Experimental, Diagnostic and Specialty Medicine University of Bologna  Bologna Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michele","family":"Cavo","sequence":"additional","affiliation":[{"name":"Department of Experimental, Diagnostic and Specialty Medicine University of Bologna  Bologna Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nicola","family":"Vianelli","sequence":"additional","affiliation":[{"name":"Department of Experimental, Diagnostic and Specialty Medicine University of Bologna  Bologna Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Francesca","family":"Palandri","sequence":"additional","affiliation":[{"name":"Department of Experimental, Diagnostic and Specialty Medicine University of Bologna  Bologna Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2017,5,16]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2009-03-209262"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2007-05-091850"},{"issue":"8","key":"e_1_2_8_4_1","first-page":"911","article-title":"Value of bone marrow biopsy in the diagnosis of essential thrombocythemia","volume":"89","author":"Florena AM","year":"2004","journal-title":"Haematologica"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1080\/10428190600678975"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01695473"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1159\/000083452"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2012-01-407981"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1038\/leu.2015.87"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2010.34.5298"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.11653"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.thromres.2009.02.004"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/cncr.22663"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12032-012-0190-3"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.12053"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2012-07-444067"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1208500"},{"key":"e_1_2_8_18_1","volume-title":"Tumors of Haematopoietic and Lymphoid Tissues","author":"Jaffe E","year":"2001"},{"key":"e_1_2_8_19_1","volume-title":"al e. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues","author":"Swerdlow S","year":"2008"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.leu.2404914"},{"key":"e_1_2_8_21_1","doi-asserted-by":"publisher","DOI":"10.1038\/modpathol.2012.87"},{"key":"e_1_2_8_22_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.23585"},{"key":"e_1_2_8_23_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.23617"},{"key":"e_1_2_8_24_1","doi-asserted-by":"publisher","DOI":"10.1038\/leu.2013.219"},{"key":"e_1_2_8_25_1","doi-asserted-by":"publisher","DOI":"10.1080\/10428190802688152"},{"key":"e_1_2_8_26_1","volume-title":"ISCN 2009: International System of Human Cytogenetic Nomenclature","author":"Shaffer LG","year":"2009"},{"key":"e_1_2_8_27_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2009.24.5233"},{"key":"e_1_2_8_28_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.21360"},{"key":"e_1_2_8_29_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2006-12-064287"},{"key":"e_1_2_8_30_1","doi-asserted-by":"publisher","DOI":"10.1038\/leu.2010.148"},{"key":"e_1_2_8_31_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V96.13.4261"},{"key":"e_1_2_8_32_1","doi-asserted-by":"publisher","DOI":"10.1034\/j.1600-0609.2001.00359.x"},{"key":"e_1_2_8_33_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.exphem.2005.01.015"},{"issue":"2","key":"e_1_2_8_34_1","first-page":"169","article-title":"Increased platelet and leukocyte activation as contributing mechanisms for thrombosis in essential thrombocythemia and correlation with the JAK2 mutational status","volume":"91","author":"Arellano\u2010Rodrigo E","year":"2006","journal-title":"Haematologica"},{"key":"e_1_2_8_35_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2006-09-046342"},{"key":"e_1_2_8_36_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00277-010-1154-3"},{"key":"e_1_2_8_37_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2012-07-442012"}],"container-title":["Hematological Oncology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fhon.2430","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/hon.2430","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,24]],"date-time":"2023-09-24T10:54:04Z","timestamp":1695552844000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/hon.2430"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,5,16]]},"references-count":36,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2018,2]]}},"alternative-id":["10.1002\/hon.2430"],"URL":"https:\/\/doi.org\/10.1002\/hon.2430","archive":["Portico"],"relation":{},"ISSN":["0278-0232","1099-1069"],"issn-type":[{"value":"0278-0232","type":"print"},{"value":"1099-1069","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017,5,16]]}}}