Author/Authors :
Yavarian ، M نويسنده Haemostasis and Thrombosis Unit, Haematology Research Center, Shiraz University of Medical Sciences, Iran , , Farsheedfar ، GR نويسنده The Thalassemia Medical Centre, Medical Faculty, Bandar Abbas University, Iran , , Karimi ، M نويسنده Haemostasis and Thrombosis Unit, Haematology Research Center, Shiraz University of Medical Sciences, Iran , , Almoazzez ، M نويسنده Haemostasis and Thrombosis Unit, Haematology Research Center, Shiraz University of Medical Sciences, Iran , , Harteveld ، CL نويسنده The Haemoglobinopathiees Laboratory, Dept. of Human and Clinical Genetics, Leiden University Medical Center, the Netherlands , , Giordano ، PC نويسنده The Haemoglobinopathiees Laboratory, Dept. of Human and Clinical Genetics, Leiden University Medical Center, the Netherlands ,
Abstract :
Background: The classical supportive therapy for ?-thalassemia major consists of regular blood transfusion, iron-chelation therapy and specific treatment of the related complications. A balance be-tween the maintenance of the highest possible level of haemoglobin and lowest possible level of iron accumulation in heart and liver give the best chance of survival and the best quality of life.
Methods: For the first time in Iran, we report the survival chance calculated on a cohort of 101 trans-fusion dependent individuals using Kaplan-Meier analysis. Several factors were included in our study including gender, ?-globin genotype, Ferritin level, blood group and access to transfusion, socioeco-nomic status and type of transfusion protocol.
Results: The survival rate was observed in the first decade of life. While life expectancy up to the age is satisfactory, our study is focused on the decline observed in the second decade of life. Our data shows that 68% of the patients reaches 20 years of age and that prospectively only 50% will still be alive at age 30.
Conclusion: Although information and transfusion protocols play a role, the most important factors influencing the survival rate observed in this study are the ferritin level and the molecular back-ground
