{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,25]],"date-time":"2026-01-25T23:54:38Z","timestamp":1769385278559,"version":"3.49.0"},"reference-count":41,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2016,10,17]],"date-time":"2016-10-17T00:00:00Z","timestamp":1476662400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"Foundation Franco"},{"name":"Piera Cutino"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Br J Haematol"],"published-print":{"date-parts":[[2017,1]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>In the last few decades, the life expectancy of regularly transfused \u03b2\u2010thalassaemia major (<jats:styled-content style=\"fixed-case\">TM<\/jats:styled-content>) patients has dramatically improved following the introduction of safe transfusion practices, iron chelation therapy, aggressive treatment of infections and improved management of cardiac complications. How such changes, especially those attributed to the introduction of iron chelation therapy, improved the survival of <jats:styled-content style=\"fixed-case\">TM<\/jats:styled-content> patients to approach those with \u03b2\u2010thalassaemia intermedia (<jats:styled-content style=\"fixed-case\">TI<\/jats:styled-content>) remains unknown. Three hundred and seventy\u2010nine patients with <jats:styled-content style=\"fixed-case\">TM<\/jats:styled-content> (<jats:italic>n<\/jats:italic>\u00a0=\u00a0284, dead 40) and <jats:styled-content style=\"fixed-case\">TI<\/jats:styled-content> (<jats:italic>n<\/jats:italic>\u00a0=\u00a095, dead 13) were followed retrospectively since birth until 30 June 2015 or death. Kaplan\u2010Meir curves showed statistically significant differences in <jats:styled-content style=\"fixed-case\">TM<\/jats:styled-content> and <jats:styled-content style=\"fixed-case\">TI<\/jats:styled-content> survival (<jats:italic>P\u00a0<\/jats:italic>&lt;<jats:italic>\u00a0<\/jats:italic>0\u00b70001) before the introduction of iron chelation in 1965, which were no longer apparent after that date (<jats:italic>P\u00a0<\/jats:italic>=<jats:italic>\u00a0<\/jats:italic>0\u00b7086), reducing the Hazard Ratio of death in <jats:styled-content style=\"fixed-case\">TM<\/jats:styled-content> compared to <jats:styled-content style=\"fixed-case\">TI<\/jats:styled-content> from 6\u00b78 [95% confidence interval (<jats:styled-content style=\"fixed-case\">CI<\/jats:styled-content>) 2\u00b76\u201317\u00b75] before 1965 to 2\u00b78 (95% <jats:styled-content style=\"fixed-case\">CI<\/jats:styled-content> 0\u00b78\u20139\u00b72). These findings suggest that, in the era of iron chelation therapy and improved survival for <jats:styled-content style=\"fixed-case\">TM<\/jats:styled-content>, the major\u2010intermedia dichotomy needs to be revisited alongside future directions in general management and prevention for both conditions.<\/jats:p>","DOI":"10.1111\/bjh.14381","type":"journal-article","created":{"date-parts":[[2016,10,17]],"date-time":"2016-10-17T10:53:18Z","timestamp":1476701598000},"page":"124-130","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":48,"title":["The era of comparable life expectancy between thalassaemia major and intermedia: Is it time to revisit the major\u2010intermedia dichotomy?"],"prefix":"10.1111","volume":"176","author":[{"given":"Angela","family":"Vitrano","sequence":"first","affiliation":[{"name":"Campus of Haematology Franco e Piera Cutino \u2010 A.O.O.R. \u201cVilla Sofia\u2010Cervello\u201d Palermo Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Giuseppina","family":"Calvaruso","sequence":"additional","affiliation":[{"name":"Campus of Haematology Franco e Piera Cutino \u2010 A.O.O.R. \u201cVilla Sofia\u2010Cervello\u201d Palermo Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Eliana","family":"Lai","sequence":"additional","affiliation":[{"name":"Ospedale microcitemico Centro Talassemici Adulti University of Cagliari Cagliari Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Grazia","family":"Colletta","sequence":"additional","affiliation":[{"name":"Azienda Ospedaliera Universit\u00e0 Policlinico \u201cVittorio Emanuele \u2010 P.O. Ferrarotto\u201d Catania Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alessandra","family":"Quota","sequence":"additional","affiliation":[{"name":"U.O.S Talassemia P.O. Vittorio Emanuele Gela (CL) Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Calogera","family":"Gerardi","sequence":"additional","affiliation":[{"name":"U.O.S. di Talassemia P.O. \u201cGiovanni Paolo II\u201d Sciacca Sciacca Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Luciana","family":"Concetta Rigoli","sequence":"additional","affiliation":[{"name":"U.O. Genetica ed Immunologia Pediatrica Policlinico \u201cG. Martino\u201d Messina Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lorella","family":"Pitrolo","sequence":"additional","affiliation":[{"name":"Campus of Haematology Franco e Piera Cutino \u2010 A.O.O.R. \u201cVilla Sofia\u2010Cervello\u201d Palermo Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Liana","family":"Cuccia","sequence":"additional","affiliation":[{"name":"U.O.C. 7<sup>a<\/sup> Pediatria per le Emopatie Ereditarie Centro di Prevenzione Diagnosi e Cura della Talassemia A.O. \u201cG. Di Cristina\u201d Palermo Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Francesco","family":"Gagliardotto","sequence":"additional","affiliation":[{"name":"U.O.C. 7<sup>a<\/sup> Pediatria per le Emopatie Ereditarie Centro di Prevenzione Diagnosi e Cura della Talassemia A.O. \u201cG. Di Cristina\u201d Palermo Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Aldo","family":"Filosa","sequence":"additional","affiliation":[{"name":"U.O.D. Malattie Rare del Globulo Rosso A.O.R.N. \u201cA. Cardarelli\u201d Napoli Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Vincenzo","family":"Caruso","sequence":"additional","affiliation":[{"name":"U.O. Talassemia Ospedale Garibaldi Catania Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Crocetta","family":"Argento","sequence":"additional","affiliation":[{"name":"U.O.C. Medicina Trasfusionale A.O. \u201cS. Giovanni di Dio\u201d Agrigento Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Saveria","family":"Campisi","sequence":"additional","affiliation":[{"name":"U.O.S. Talassemia A.O. 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