{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,17]],"date-time":"2026-10-17T00:53:38Z","timestamp":1792198418010,"version":"4.4.0"},"reference-count":31,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2020,1,16]],"date-time":"2020-01-16T00:00:00Z","timestamp":1579132800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100000871","name":"Mayo Clinic","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000871","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100004434","name":"Universit\u00e0 degli Studi di Firenze","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100004434","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100005010","name":"Associazione Italiana per la Ricerca sul Cancro","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100005010","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003196","name":"Ministero della Salute","doi-asserted-by":"publisher","award":["GR\u20102011\u201002352109"],"award-info":[{"award-number":["GR\u20102011\u201002352109"]}],"id":[{"id":"10.13039\/501100003196","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Br J Haematol"],"published-print":{"date-parts":[[2020,4]]},"abstract":"<jats:title>Summary<\/jats:title>\n                  <jats:p>\n                    Survival prediction in essential thrombocythaemia (ET) and polycythaemia vera (PV) is currently based on clinically\u2010derived variables; we examined the possibility of integrating genetic information for predicting survival. To this end, 906 molecularly\u2010annotated patients (416 Mayo Clinic; 490 University of Florence, Italy), including 502 ET and 404 PV, were recruited. Multivariable analysis identified spliceosome mutations to adversely affect overall (\n                    <jats:italic>SF3B1<\/jats:italic>\n                    ,\n                    <jats:italic>SRSF2<\/jats:italic>\n                    in ET and\n                    <jats:italic>SRSF2<\/jats:italic>\n                    in PV) and myelofibrosis\u2010free (\n                    <jats:italic>U2AF1<\/jats:italic>\n                    ,\n                    <jats:italic>SF3B1<\/jats:italic>\n                    in ET) survival;\n                    <jats:italic>TP53<\/jats:italic>\n                    mutations predicted leukaemic transformation in ET; \u201cadverse\u201d mutations occurred in 51 (10%) ET and 8 (2%) PV patients. We confirmed the independent survival effect of adverse mutations [hazard ratio (HR) 2\u00b74, 95% CI 1\u00b76\u20133\u00b75], age &gt;60\u00a0years (6\u00b76, 4\u00b76\u20139\u00b77), male sex (1\u00b78, 1\u00b73\u20132\u00b74) and leukocytosis \u226511\u00a0\u00d7\u00a010\n                    <jats:sup>9<\/jats:sup>\n                    \/l (1\u00b76, 1\u00b71\u20132\u00b72), in ET, and adverse mutations (7\u00b78, 3\u00b71\u201317\u00b70), age &gt;67\u00a0years (5\u00b74, 3\u00b76\u20138\u00b71), leukocytosis \u226515\u00a0\u00d7\u00a010\n                    <jats:sup>9<\/jats:sup>\n                    \/l (2\u00b78, 1\u00b78\u20134\u00b72) and thrombosis history (2\u00b70, 1\u00b74\u20132\u00b79), in PV. HR\u2010based risk point allocation allowed development of three\u2010tiered mutation\u2010enhanced international prognostic systems (MIPSS) which were validated in both cohorts and performance was shown to be superior to conventional scoring systems. Spliceosome mutations enhance survival prediction in ET and PV and identify patients at risk for fibrotic progression.\n                    <jats:italic>TP53<\/jats:italic>\n                    mutations predict leukaemic transformation in ET.\n                  <\/jats:p>","DOI":"10.1111\/bjh.16380","type":"journal-article","created":{"date-parts":[[2020,1,16]],"date-time":"2020-01-16T18:39:20Z","timestamp":1579199960000},"page":"291-302","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":222,"title":["Mutation\u2010enhanced international prognostic systems for essential thrombocythaemia and polycythaemia vera"],"prefix":"10.1111","volume":"189","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4605-3821","authenticated-orcid":false,"given":"Ayalew","family":"Tefferi","sequence":"first","affiliation":[{"name":"Division of Hematology Departments of Internal Medicine and Laboratory Medicine Mayo Clinic Rochester MN 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Universitaria Careggi University of Florence, and Denothe Excellence Center Florence Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christy M.","family":"Finke","sequence":"additional","affiliation":[{"name":"Division of Hematology Departments of Internal Medicine and Laboratory Medicine Mayo Clinic Rochester MN USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giuseppe G.","family":"Loscocco","sequence":"additional","affiliation":[{"name":"Department of Experimental and Clinical Medicine CRIMM Center of Research and Innovation of Myeloproliferative Neoplasms Azienda Ospedaliera Universitaria Careggi University of Florence, and Denothe Excellence Center Florence Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Benedetta","family":"Sordi","sequence":"additional","affiliation":[{"name":"Department of Experimental and Clinical Medicine CRIMM Center of Research and Innovation of Myeloproliferative Neoplasms Azienda Ospedaliera Universitaria Careggi University of Florence, and Denothe Excellence Center Florence Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Natasha","family":"Szuber","sequence":"additional","affiliation":[{"name":"Division of Hematology Departments of Internal Medicine and Laboratory Medicine Mayo Clinic Rochester MN USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giada","family":"Rotunno","sequence":"additional","affiliation":[{"name":"Department of Experimental and Clinical Medicine CRIMM Center of Research and Innovation of Myeloproliferative Neoplasms Azienda Ospedaliera Universitaria Careggi University of Florence, and Denothe Excellence Center Florence Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annalisa","family":"Pacilli","sequence":"additional","affiliation":[{"name":"Department of Experimental and Clinical Medicine CRIMM Center of Research and Innovation of Myeloproliferative Neoplasms Azienda 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