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
Main Authors: 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
Format: Article
Language:English
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
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Summary: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 
ISSN:0007-1048
1365-2141
DOI:10.1111/bjh.14381