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The era of comparable life expectancy between thalassaemia major and intermedia: Is it time to revisit the major‐intermedia dichotomy?
Author(s) -
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
Publication year - 2017
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/bjh.14381
Subject(s) - chelation therapy , intermedia , medicine , hazard ratio , life expectancy , confidence interval , thalassemia , deferiprone , beta thalassemia , survival analysis , pediatrics , history , population , environmental health , performance art , art history
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.