TY - JOUR
TI - Survival in a large cohort of Greek patients with transfusion-dependent beta thalassaemia and mortality ratios compared to the general population
AU - Ladis, V.
AU - Chouliaras, G.
AU - Berdoukas, V.
AU - Chatziliami, A.
AU - Fragodimitri, C.
AU - Karabatsos, F.
AU - Youssef, J.
AU - Kattamis, A.
AU - Karagiorga-Lagana, M.
JO - European Journal of Haematology
PY - 2011
VL - 86
TODO - 4
SP - 332-338
PB - 
SN - 0902-4441, 1600-0609
TODO - 10.1111/j.1600-0609.2011.01582.x
TODO - deferasirox;  deferiprone;  deferoxamine, accident;  adult;  age distribution;  aged;  article;  beta thalassemia;  birth;  controlled study;  disease free survival;  female;  Greece;  heart death;  heart disease;  human;  Human immunodeficiency virus infection;  liver disease;  major clinical study;  male;  mortality;  overall survival;  priority journal;  prospective study;  risk assessment;  septicemia;  sex difference;  stem cell transplantation;  survival;  thromboembolism;  transfusion dependent beta thalassemia, Adolescent;  Adult;  beta-Thalassemia;  Blood Transfusion;  Cause of Death;  Child;  Child, Preschool;  Cohort Studies;  Female;  Greece;  Humans;  Infant;  Infant, Newborn;  Kaplan-Meier Estimate;  Male;  Middle Aged;  Prospective Studies;  Young Adult
TODO - 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 50years, 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 20-40years) 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. © 2011 John Wiley & Sons A/S.
ER -