BACKGROUND AND OBJECTIVES: Seven Italian centers reported data on survival, causes of death and appearance of complications in patients with thalassemia major. The interactions between gender, birth cohort, complications, and ferritin on survival and complications were analyzed. DESIGN AND METHODS: Survival after the first decade was studied for 977 patients born since 1960 whereas survival since birth and complication appearance was studied for the 720 patients born after 1970. Better survival was demonstrated for patients born in more recent years (p<0.00005) and for females (p=0.0003); 68% of the patients are alive at the age of 35 years. In the entire population 67% of the deaths were due to heart disease. RESULTS: There was a significant association between birth cohort and complication-free survival (p<0.0005). The prevalence of complications was: heart failure 6.8%, arrhythmia 5.7%, hypogonadism 54.7%, hypothyroidism 10.8%, diabetes 6.4%, HIV infection 1.7%, and thrombosis 1.1%. Lower ferritin levels were associated with a lower probability of heart failure (hazard ratio =3.35, p<0.005) and with prolonged survival (hazard ratio = 2.45, p<0.005), using a cut-off as low as 1,000 ng/mL. INTERPRETATION AND CONCLUSIONS: Survival and complication-free survival of patients with thalassemia major continue to improve, especially for female patients born shortly before or after the availability of iron chelation.
BACKGROUND AND OBJECTIVES: Seven Italian centers reported data on survival, causes of death and appearance of complications in patients with thalassemia major. The interactions between gender, birth cohort, complications, and ferritin on survival and complications were analyzed. DESIGN AND METHODS: Survival after the first decade was studied for 977 patients born since 1960 whereas survival since birth and complication appearance was studied for the 720 patients born after 1970. Better survival was demonstrated for patients born in more recent years (p<0.00005) and for females (p=0.0003); 68% of the patients are alive at the age of 35 years. In the entire population 67% of the deaths were due to heart disease. RESULTS: There was a significant association between birth cohort and complication-free survival (p<0.0005). The prevalence of complications was: heart failure 6.8%, arrhythmia 5.7%, hypogonadism 54.7%, hypothyroidism 10.8%, diabetes 6.4%, HIV infection 1.7%, and thrombosis 1.1%. Lower ferritin levels were associated with a lower probability of heart failure (hazard ratio =3.35, p<0.005) and with prolonged survival (hazard ratio = 2.45, p<0.005), using a cut-off as low as 1,000 ng/mL. INTERPRETATION AND CONCLUSIONS: Survival and complication-free survival of patients with thalassemia major continue to improve, especially for female patients born shortly before or after the availability of iron chelation.
Authors: Haris Riaz; Talha Riaz; Muhammad Ubaid Khan; Sina Aziz; Faizan Ullah; Anis Rehman; Qandeel Zafar; Abdul Nafey Kazi Journal: BMC Res Notes Date: 2011-08-11
Authors: Louise de Swart; Jan C M Hendriks; Lisa N van der Vorm; Z Ioav Cabantchik; Patricia J Evans; Eldad A Hod; Gary M Brittenham; Yael Furman; Boguslaw Wojczyk; Mirian C H Janssen; John B Porter; Vera E J M Mattijssen; Bart J Biemond; Marius A MacKenzie; Raffaella Origa; Renzo Galanello; Robert C Hider; Dorine W Swinkels Journal: Haematologica Date: 2015-09-18 Impact factor: 9.941
Authors: Janet L Kwiatkowski; Hae-Young Kim; Alexis A Thompson; Charles T Quinn; Brigitta U Mueller; Isaac Odame; Patricia J Giardina; Elliott P Vichinsky; Jeanne M Boudreaux; Alan R Cohen; John B Porter; Thomas Coates; Nancy F Olivieri; Ellis J Neufeld Journal: Blood Date: 2012-01-25 Impact factor: 22.113