Survival in a large cohort of Greek patients with transfusion-dependent beta thalassaemia and mortality ratios compared to the general population

• Published in: European journal of haematology Vol. 86; no. 4; pp. 332 - 338
• Main Authors: Ladis, Vassilis, Chouliaras, Giorgos, Berdoukas, Vasilios, Chatziliami, Antonia, Fragodimitri, Christina, Karabatsos, Fotis, Youssef, Jacqueline, Kattamis, Antonis, Karagiorga-Lagana, Markissia
• Format: Journal Article
• Language: English
• Published: Oxford, UK Blackwell Publishing Ltd 01.04.2011
• Subjects: Adolescent; Adult; beta-Thalassemia - mortality; beta-Thalassemia - therapy; Blood Transfusion; cardiac death; Cause of Death - trends; Child; Child, Preschool; Cohort Studies; Female; Greece - epidemiology; Humans; Infant; Infant, Newborn; iron overload; Kaplan-Meier Estimate; Male; Middle Aged; Prospective Studies; standardised mortality ratio; survival; transfusion-dependent beta thalassaemia; Young Adult; Greece
• ISSN: 0902-4441; 1600-0609
• DOI: 10.1111/j.1600-0609.2011.01582.x

Background:  With transfusions and chelation therapy, the prognosis for transfusion‐dependent beta thalassaemia has changed from being fatal in early childhood to a chronic disorder with prolonged survival. Design and methods:  In this historical prospective study, we present survival, causes of death and mortality ratios compared to the general population in 1044 Greek patients with transfusion‐dependent beta thalassaemia. Results:  At the age of 50 years, the overall survival was 65.0%, while the cardiac death–free survival was 77%. Birth cohort had a significant effect on survival (P < 0.001) with a negative trend towards past decades. The standardised mortality ratio (standardised for sex and ages 20–40 years) compared to the general population improved significantly from 28.9 in 1990–1999 to 13.5 in 2000–2008, while the standardised cardiac mortality ratio reduced from 322.9 to 106.6, respectively. Conclusions:  Survival in thalassaemia has dramatically improved over the last twenty years but mortality remains significantly increased, compared to the general population.