{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,5]],"date-time":"2026-09-05T14:08:15Z","timestamp":1788617295539,"version":"build-2803163510"},"reference-count":24,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2021,9,6]],"date-time":"2021-09-06T00:00:00Z","timestamp":1630886400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[2021,11]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    The current retrospective study involving a total of 1607 patients was designed to identify clinical and molecular variables that were predictive of inferior myelofibrosis\u2010free survival (MFS) in WHO\u2010defined essential thrombocythemia (ET), utilizing three independent patient cohorts: University of Florence, Italy (\n                    <jats:italic>n<\/jats:italic>\n                    \u00a0=\u2009718); Mayo Clinic, USA (\n                    <jats:italic>n<\/jats:italic>\n                    \u00a0=\u2009479) and Policlinico Gemelli, Catholic University, Rome, Italy (\n                    <jats:italic>n<\/jats:italic>\n                    \u00a0=\u2009410). The Florence patient cohort was first examined to identify independent risk factors for MFS, which included age\u2009&gt;\u200960\u2009years (HR 2.5, 95% CI 1.3\u20134.9), male sex (2.1, 1.2\u20133.9), palpable splenomegaly (2.1, 1.2\u20133.9),\n                    <jats:italic>CALR<\/jats:italic>\n                    1\/1\u2010like or\n                    <jats:italic>MPL<\/jats:italic>\n                    mutation (3.4, 1.9\u20136.1) and\n                    <jats:italic>JAK2<\/jats:italic>\n                    V617F variant allele frequency\u2009&gt;\u200935% (4.2, 1.6\u201310.8). Subsequently, an operational molecular risk category was developed and validated in the other two cohorts from Mayo Clinic and Rome: \u201chigh molecular risk\u201d category included patients with\n                    <jats:italic>JAK2<\/jats:italic>\n                    V617F VAF &gt;35%,\n                    <jats:italic>CALR<\/jats:italic>\n                    type 1\/1\u2010like or\n                    <jats:italic>MPL<\/jats:italic>\n                    mutations; all other driver mutation profiles were assigned to \u201clow molecular risk\u201d category. The former, compared to the latter molecular risk category, displayed significantly higher risk of fibrotic transformation: Florence cohort with respective fibrotic transformation risk rates of 8% vs. 1.2% at 10\u2009years and 33% vs. 8% at 20\u2009years (\n                    <jats:italic>p<\/jats:italic>\n                    \u00a0&lt;\u20090.001; HR 6.1; 95% CI 3.2\u201311.7); Mayo Cohort, 16% vs. 7% at 10\u2009years and 44% vs. 25% at 20\u2009years (\n                    <jats:italic>p<\/jats:italic>\n                    \u00a0&lt;\u20090.001; HR 2.5; 95% CI 1.6\u20134.1); and Rome cohort 7.8% vs. 4.6% at 10\u2009years and 31.2% vs. 7.1% at 20\u2009years (\n                    <jats:italic>p<\/jats:italic>\n                    \u00a0=\u20090.007, HR 2.7; 95% CI 1.3\u20135.8). The present study provides practically useful risk signals for fibrotic transformation in ET and facilitates identification of patients who require close monitoring and appropriate counseling.\n                  <\/jats:p>","DOI":"10.1002\/ajh.26332","type":"journal-article","created":{"date-parts":[[2021,8,23]],"date-time":"2021-08-23T09:16:25Z","timestamp":1629710185000},"page":"1472-1480","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":40,"title":["Clinical and molecular predictors of fibrotic progression in essential thrombocythemia: A multicenter study involving 1607 patients"],"prefix":"10.1002","volume":"96","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6241-1206","authenticated-orcid":false,"given":"Giuseppe G.","family":"Loscocco","sequence":"first","affiliation":[{"name":"Department of Experimental and Clinical Medicine, CRIMM, Center of Research and Innovation of Myeloproliferative Neoplasms, Azienda Ospedaliero\u2010Universitaria Careggi University of Florence  Florence Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1809-284X","authenticated-orcid":false,"given":"Paola","family":"Guglielmelli","sequence":"additional","affiliation":[{"name":"Department of Experimental and Clinical Medicine, CRIMM, Center of Research and Innovation of Myeloproliferative Neoplasms, Azienda Ospedaliero\u2010Universitaria Careggi University of Florence  Florence Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9104-6172","authenticated-orcid":false,"given":"Naseema","family":"Gangat","sequence":"additional","affiliation":[{"name":"Divisions of Hematology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elena","family":"Rossi","sequence":"additional","affiliation":[{"name":"Section of Hematology, Department of Radiological and Hematological Sciences Catholic University  Rome Italy"},{"name":"Fondazione Policlinico Universitario A. Gemelli IRCCS  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carmela","family":"Mannarelli","sequence":"additional","affiliation":[{"name":"Department of Experimental and Clinical Medicine, CRIMM, Center of Research and Innovation of Myeloproliferative Neoplasms, Azienda Ospedaliero\u2010Universitaria Careggi University of Florence  Florence Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Silvia","family":"Betti","sequence":"additional","affiliation":[{"name":"Fondazione Policlinico Universitario A. Gemelli IRCCS  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chiara","family":"Maccari","sequence":"additional","affiliation":[{"name":"Department of Experimental and Clinical Medicine, CRIMM, Center of Research and Innovation of Myeloproliferative Neoplasms, Azienda Ospedaliero\u2010Universitaria Careggi University of Florence  Florence Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesco","family":"Ramundo","sequence":"additional","affiliation":[{"name":"Section of Hematology, Department of Radiological and Hematological Sciences Catholic University  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yamna","family":"Jadoon","sequence":"additional","affiliation":[{"name":"Divisions of Hematology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesca","family":"Gesullo","sequence":"additional","affiliation":[{"name":"Department of Experimental and Clinical Medicine, CRIMM, Center of Research and Innovation of Myeloproliferative Neoplasms, Azienda Ospedaliero\u2010Universitaria Careggi University of Florence  Florence Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sara","family":"Ceglie","sequence":"additional","affiliation":[{"name":"Section of Hematology, Department of Radiological and Hematological Sciences Catholic University  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chiara","family":"Paoli","sequence":"additional","affiliation":[{"name":"Department of Experimental and Clinical Medicine, CRIMM, Center of Research and Innovation of Myeloproliferative Neoplasms, Azienda Ospedaliero\u2010Universitaria Careggi University of Florence  Florence Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9084-4148","authenticated-orcid":false,"given":"Animesh","family":"Pardanani","sequence":"additional","affiliation":[{"name":"Divisions of Hematology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5178-5827","authenticated-orcid":false,"given":"Valerio","family":"De Stefano","sequence":"additional","affiliation":[{"name":"Section of Hematology, Department of Radiological and Hematological Sciences Catholic University  Rome Italy"},{"name":"Fondazione Policlinico Universitario A. 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