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The era of comparable life expectancy between thalassaemia major and intermedia: Is it time to revisit the major‐intermedia dichotomy?
Summary In the last few decades, the life expectancy of regularly transfused β‐thalassaemia major (TM) patients has dramatically improved following the introduction of safe transfusion practices, iron chelation therapy, aggressive treatment of infections and improved management of cardiac complicati...
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| Published in: | British journal of haematology 2017-01, Vol.176 (1), p.124-130 |
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| container_title | British journal of haematology |
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| creator | Vitrano, Angela Calvaruso, Giuseppina Lai, Eliana Colletta, Grazia Quota, Alessandra Gerardi, Calogera Concetta Rigoli, Luciana Pitrolo, Lorella Cuccia, Liana Gagliardotto, Francesco Filosa, Aldo Caruso, Vincenzo Argento, Crocetta Campisi, Saveria Rizzo, Michele Prossomariti, Luciano Fidone, Carmelo Fustaneo, Maria Di Maggio, Rosario Maggio, Aurelio |
| description | Summary
In the last few decades, the life expectancy of regularly transfused β‐thalassaemia major (TM) 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 TM patients to approach those with β‐thalassaemia intermedia (TI) remains unknown. Three hundred and seventy‐nine patients with TM (n = 284, dead 40) and TI (n = 95, dead 13) were followed retrospectively since birth until 30 June 2015 or death. Kaplan‐Meir curves showed statistically significant differences in TM and TI survival (P |
| doi_str_mv | 10.1111/bjh.14381 |
| format | article |
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In the last few decades, the life expectancy of regularly transfused β‐thalassaemia major (TM) 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 TM patients to approach those with β‐thalassaemia intermedia (TI) remains unknown. Three hundred and seventy‐nine patients with TM (n = 284, dead 40) and TI (n = 95, dead 13) were followed retrospectively since birth until 30 June 2015 or death. Kaplan‐Meir curves showed statistically significant differences in TM and TI survival (P < 0·0001) before the introduction of iron chelation in 1965, which were no longer apparent after that date (P = 0·086), reducing the Hazard Ratio of death in TM compared to TI from 6·8 [95% confidence interval (CI) 2·6–17·5] before 1965 to 2·8 (95% CI 0·8–9·2). These findings suggest that, in the era of iron chelation therapy and improved survival for TM, the major‐intermedia dichotomy needs to be revisited alongside future directions in general management and prevention for both conditions.</description><identifier>ISSN: 0007-1048</identifier><identifier>EISSN: 1365-2141</identifier><identifier>DOI: 10.1111/bjh.14381</identifier><identifier>PMID: 27748513</identifier><language>eng</language><publisher>England: Blackwell Publishing Ltd</publisher><subject>Adult ; beta-Thalassemia - classification ; beta-Thalassemia - epidemiology ; beta-Thalassemia - mortality ; beta-Thalassemia - therapy ; Blood diseases ; Chelation ; Chelation therapy ; classification ; Complications ; Female ; Follow-Up Studies ; Heart diseases ; Hematology ; Humans ; Iron ; Lead poisoning ; Life Expectancy ; Life span ; Male ; Middle Aged ; Retrospective Studies ; severity ; Statistical analysis ; Survival ; Survival Rate ; thalassaemia ; Transfusion ; Young Adult</subject><ispartof>British journal of haematology, 2017-01, Vol.176 (1), p.124-130</ispartof><rights>2016 John Wiley & Sons Ltd</rights><rights>2016 John Wiley & Sons Ltd.</rights><rights>Copyright © 2017 John Wiley & Sons Ltd</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c4211-996101515c0d03e4e5426e7a00fd4e79403f9e4237042eeda12b763e7cd2b2ad3</citedby><cites>FETCH-LOGICAL-c4211-996101515c0d03e4e5426e7a00fd4e79403f9e4237042eeda12b763e7cd2b2ad3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,780,784,27924,27925</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/27748513$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Vitrano, Angela</creatorcontrib><creatorcontrib>Calvaruso, Giuseppina</creatorcontrib><creatorcontrib>Lai, Eliana</creatorcontrib><creatorcontrib>Colletta, Grazia</creatorcontrib><creatorcontrib>Quota, Alessandra</creatorcontrib><creatorcontrib>Gerardi, Calogera</creatorcontrib><creatorcontrib>Concetta Rigoli, Luciana</creatorcontrib><creatorcontrib>Pitrolo, Lorella</creatorcontrib><creatorcontrib>Cuccia, Liana</creatorcontrib><creatorcontrib>Gagliardotto, Francesco</creatorcontrib><creatorcontrib>Filosa, Aldo</creatorcontrib><creatorcontrib>Caruso, Vincenzo</creatorcontrib><creatorcontrib>Argento, Crocetta</creatorcontrib><creatorcontrib>Campisi, Saveria</creatorcontrib><creatorcontrib>Rizzo, Michele</creatorcontrib><creatorcontrib>Prossomariti, Luciano</creatorcontrib><creatorcontrib>Fidone, Carmelo</creatorcontrib><creatorcontrib>Fustaneo, Maria</creatorcontrib><creatorcontrib>Di Maggio, Rosario</creatorcontrib><creatorcontrib>Maggio, Aurelio</creatorcontrib><title>The era of comparable life expectancy between thalassaemia major and intermedia: Is it time to revisit the major‐intermedia dichotomy?</title><title>British journal of haematology</title><addtitle>Br J Haematol</addtitle><description>Summary
In the last few decades, the life expectancy of regularly transfused β‐thalassaemia major (TM) 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 TM patients to approach those with β‐thalassaemia intermedia (TI) remains unknown. Three hundred and seventy‐nine patients with TM (n = 284, dead 40) and TI (n = 95, dead 13) were followed retrospectively since birth until 30 June 2015 or death. Kaplan‐Meir curves showed statistically significant differences in TM and TI survival (P < 0·0001) before the introduction of iron chelation in 1965, which were no longer apparent after that date (P = 0·086), reducing the Hazard Ratio of death in TM compared to TI from 6·8 [95% confidence interval (CI) 2·6–17·5] before 1965 to 2·8 (95% CI 0·8–9·2). These findings suggest that, in the era of iron chelation therapy and improved survival for TM, the major‐intermedia dichotomy needs to be revisited alongside future directions in general management and prevention for both conditions.</description><subject>Adult</subject><subject>beta-Thalassemia - classification</subject><subject>beta-Thalassemia - epidemiology</subject><subject>beta-Thalassemia - mortality</subject><subject>beta-Thalassemia - therapy</subject><subject>Blood diseases</subject><subject>Chelation</subject><subject>Chelation therapy</subject><subject>classification</subject><subject>Complications</subject><subject>Female</subject><subject>Follow-Up Studies</subject><subject>Heart diseases</subject><subject>Hematology</subject><subject>Humans</subject><subject>Iron</subject><subject>Lead poisoning</subject><subject>Life Expectancy</subject><subject>Life span</subject><subject>Male</subject><subject>Middle Aged</subject><subject>Retrospective Studies</subject><subject>severity</subject><subject>Statistical analysis</subject><subject>Survival</subject><subject>Survival Rate</subject><subject>thalassaemia</subject><subject>Transfusion</subject><subject>Young Adult</subject><issn>0007-1048</issn><issn>1365-2141</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2017</creationdate><recordtype>article</recordtype><recordid>eNqN0U9rFDEYBvAgFrtWD34BCXjRw7R582ey00uxpdpKwUs9h0zmHTbLzGRNsm335tGjn9FP0rRbKwgFcwkJP56X5CHkDbB9KOugXS72QYo5PCMzELWqOEh4TmaMMV0Bk_Nd8jKlJWMgmIIXZJdrLecKxIz8vFwgxWhp6KkL48pG2w5IB9-X65sVumwnt6Et5mvEieaFHWxKFkdv6WiXIVI7ddRPGeOInbeH9DxRn2n2I9IcaMQrn-7OZcy9__3j119NO-8WIYdxc_SK7PR2SPj6Yd8j3z6dXp6cVRdfP5-ffLyonOQAVdPUwECBcqxjAiUqyWvUlrG-k6gbyUTfoORCM8kROwu81bVA7TrectuJPfJ-m7uK4fsaUzajTw6HwU4Y1snAXDVSS-DyP6hQUjRlVKHv_qHLsI5TeYiBhjOpGq7qoj5slYshpYi9WUU_2rgxwMxdk6Y0ae6bLPbtQ-K6LX_1KP9UV8DBFlz7ATdPJ5njL2fbyFtuhKhM</recordid><startdate>201701</startdate><enddate>201701</enddate><creator>Vitrano, Angela</creator><creator>Calvaruso, Giuseppina</creator><creator>Lai, Eliana</creator><creator>Colletta, Grazia</creator><creator>Quota, Alessandra</creator><creator>Gerardi, Calogera</creator><creator>Concetta Rigoli, Luciana</creator><creator>Pitrolo, Lorella</creator><creator>Cuccia, Liana</creator><creator>Gagliardotto, Francesco</creator><creator>Filosa, Aldo</creator><creator>Caruso, Vincenzo</creator><creator>Argento, Crocetta</creator><creator>Campisi, Saveria</creator><creator>Rizzo, Michele</creator><creator>Prossomariti, Luciano</creator><creator>Fidone, Carmelo</creator><creator>Fustaneo, Maria</creator><creator>Di Maggio, Rosario</creator><creator>Maggio, Aurelio</creator><general>Blackwell Publishing Ltd</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7T5</scope><scope>H94</scope><scope>7X8</scope></search><sort><creationdate>201701</creationdate><title>The era of comparable life expectancy between thalassaemia major and intermedia: Is it time to revisit the major‐intermedia dichotomy?</title><author>Vitrano, Angela ; Calvaruso, Giuseppina ; Lai, Eliana ; Colletta, Grazia ; Quota, Alessandra ; Gerardi, Calogera ; Concetta Rigoli, Luciana ; Pitrolo, Lorella ; Cuccia, Liana ; Gagliardotto, Francesco ; Filosa, Aldo ; Caruso, Vincenzo ; Argento, Crocetta ; Campisi, Saveria ; Rizzo, Michele ; Prossomariti, Luciano ; Fidone, Carmelo ; Fustaneo, Maria ; Di Maggio, Rosario ; Maggio, Aurelio</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c4211-996101515c0d03e4e5426e7a00fd4e79403f9e4237042eeda12b763e7cd2b2ad3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2017</creationdate><topic>Adult</topic><topic>beta-Thalassemia - classification</topic><topic>beta-Thalassemia - epidemiology</topic><topic>beta-Thalassemia - mortality</topic><topic>beta-Thalassemia - therapy</topic><topic>Blood diseases</topic><topic>Chelation</topic><topic>Chelation therapy</topic><topic>classification</topic><topic>Complications</topic><topic>Female</topic><topic>Follow-Up Studies</topic><topic>Heart diseases</topic><topic>Hematology</topic><topic>Humans</topic><topic>Iron</topic><topic>Lead poisoning</topic><topic>Life Expectancy</topic><topic>Life span</topic><topic>Male</topic><topic>Middle Aged</topic><topic>Retrospective Studies</topic><topic>severity</topic><topic>Statistical analysis</topic><topic>Survival</topic><topic>Survival Rate</topic><topic>thalassaemia</topic><topic>Transfusion</topic><topic>Young Adult</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Vitrano, Angela</creatorcontrib><creatorcontrib>Calvaruso, Giuseppina</creatorcontrib><creatorcontrib>Lai, Eliana</creatorcontrib><creatorcontrib>Colletta, Grazia</creatorcontrib><creatorcontrib>Quota, Alessandra</creatorcontrib><creatorcontrib>Gerardi, Calogera</creatorcontrib><creatorcontrib>Concetta Rigoli, Luciana</creatorcontrib><creatorcontrib>Pitrolo, Lorella</creatorcontrib><creatorcontrib>Cuccia, Liana</creatorcontrib><creatorcontrib>Gagliardotto, Francesco</creatorcontrib><creatorcontrib>Filosa, Aldo</creatorcontrib><creatorcontrib>Caruso, Vincenzo</creatorcontrib><creatorcontrib>Argento, Crocetta</creatorcontrib><creatorcontrib>Campisi, Saveria</creatorcontrib><creatorcontrib>Rizzo, Michele</creatorcontrib><creatorcontrib>Prossomariti, Luciano</creatorcontrib><creatorcontrib>Fidone, Carmelo</creatorcontrib><creatorcontrib>Fustaneo, Maria</creatorcontrib><creatorcontrib>Di Maggio, Rosario</creatorcontrib><creatorcontrib>Maggio, Aurelio</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Immunology Abstracts</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>MEDLINE - Academic</collection><jtitle>British journal of haematology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Vitrano, Angela</au><au>Calvaruso, Giuseppina</au><au>Lai, Eliana</au><au>Colletta, Grazia</au><au>Quota, Alessandra</au><au>Gerardi, Calogera</au><au>Concetta Rigoli, Luciana</au><au>Pitrolo, Lorella</au><au>Cuccia, Liana</au><au>Gagliardotto, Francesco</au><au>Filosa, Aldo</au><au>Caruso, Vincenzo</au><au>Argento, Crocetta</au><au>Campisi, Saveria</au><au>Rizzo, Michele</au><au>Prossomariti, Luciano</au><au>Fidone, Carmelo</au><au>Fustaneo, Maria</au><au>Di Maggio, Rosario</au><au>Maggio, Aurelio</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>The era of comparable life expectancy between thalassaemia major and intermedia: Is it time to revisit the major‐intermedia dichotomy?</atitle><jtitle>British journal of haematology</jtitle><addtitle>Br J Haematol</addtitle><date>2017-01</date><risdate>2017</risdate><volume>176</volume><issue>1</issue><spage>124</spage><epage>130</epage><pages>124-130</pages><issn>0007-1048</issn><eissn>1365-2141</eissn><abstract>Summary
In the last few decades, the life expectancy of regularly transfused β‐thalassaemia major (TM) 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 TM patients to approach those with β‐thalassaemia intermedia (TI) remains unknown. Three hundred and seventy‐nine patients with TM (n = 284, dead 40) and TI (n = 95, dead 13) were followed retrospectively since birth until 30 June 2015 or death. Kaplan‐Meir curves showed statistically significant differences in TM and TI survival (P < 0·0001) before the introduction of iron chelation in 1965, which were no longer apparent after that date (P = 0·086), reducing the Hazard Ratio of death in TM compared to TI from 6·8 [95% confidence interval (CI) 2·6–17·5] before 1965 to 2·8 (95% CI 0·8–9·2). These findings suggest that, in the era of iron chelation therapy and improved survival for TM, the major‐intermedia dichotomy needs to be revisited alongside future directions in general management and prevention for both conditions.</abstract><cop>England</cop><pub>Blackwell Publishing Ltd</pub><pmid>27748513</pmid><doi>10.1111/bjh.14381</doi><tpages>7</tpages><oa>free_for_read</oa></addata></record> |
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| ispartof | British journal of haematology, 2017-01, Vol.176 (1), p.124-130 |
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| source | Wiley-Blackwell Read & Publish Collection |
| subjects | Adult beta-Thalassemia - classification beta-Thalassemia - epidemiology beta-Thalassemia - mortality beta-Thalassemia - therapy Blood diseases Chelation Chelation therapy classification Complications Female Follow-Up Studies Heart diseases Hematology Humans Iron Lead poisoning Life Expectancy Life span Male Middle Aged Retrospective Studies severity Statistical analysis Survival Survival Rate thalassaemia Transfusion Young Adult |
| title | The era of comparable life expectancy between thalassaemia major and intermedia: Is it time to revisit the major‐intermedia dichotomy? |
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