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.
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.
Authors: Venée N Tubman; Ellen B Fung; Maria Vogiatzi; Alexis A Thompson; Zora R Rogers; Ellis J Neufeld; Janet L Kwiatkowski Journal: J Pediatr Hematol Oncol Date: 2015-04 Impact factor: 1.289
Authors: Farrukh Shah; Paul Telfer; Mark Velangi; Shivan Pancham; Robert Wynn; Sally Pollard; Elizabeth Chalmers; Jonathan Kell; Angela M Carter; Joe Hickey; Clark Paramore; Minesh Jobanputra; Kate Ryan Journal: EJHaem Date: 2021-09-08
Authors: Anuraag R Kansal; Odette S Reifsnider; Sarah B Brand; Neil Hawkins; Anna Coughlan; Shujun Li; Lael Cragin; Clark Paramore; Andrew C Dietz; J Jaime Caro Journal: J Mark Access Health Policy Date: 2021-06-07