The survival rate of patients with beta major thalassemia and thalassemia intermedia and its trend in recent years in Iran.

Objectives: Thalassemia is a common genetic disease in Iran, especially in the north and south of the country. The present study seeks to determine the survival rate of patients with thalassemia in high-endemic regions of Iran and its variation in patients born before and after 1971.

Methods: The present historical cohort study examined the health records of patients with major, intermedia and sickle beta thalassemia who had presented to thalassemia centers in the past years and specified their date of death and duration of survival. The data collected were analyzed using the study checklist and the Kaplan-Meier test, the log-rank test and the Chi-square test.

Results: A total of 5491 records were examined in this study, 2647 of which belonged to men and 2634 to women. The mean overall survival was 52.42 years, and the mean survival in cases of major, intermedia and sickle beta thalassemia was 56.59, 53.1 and 50.07 years, respectively. The risk of thalassemia major was 4.22 (P=0.04) and the risk of thalassemia intermedia 0.77 (P=0.7).

Conclusion: Compared to similar studies, the present study shows a higher survival rate in patients with thalassemia. However, comparing the survival of patients born before 1971 and those born after this year does not show any improvements. The survival of patients was unfavorable in poorer regions (Zahedan and Iranshahr). Factors including the female gender, a higher level of education, being married and living in metropolises obviate the risk of death at younger ages and improve survival.