Abstract
OBJECTIVESThalassemia is a common genetic disease in Iran, especially in the north and south of Iran. The present study sought to determine the survival rate of patients with thalassemia in highly endemic regions of Iran and its variation in patients born before and after 1971.METHODSThe present historical cohort study extracted data from the health records of patients with beta-thalassemia major, beta-thalassemia intermedia, and sickle beta-thalassemia who had presented to thalassemia treatment centers in the past years. The collected data were analyzed using the Kaplan-Meier test, the log-rank test, and the chi-square test.RESULTSOf the total of 5,491 medical records (2,647 men and 2,634 women; mean age, 23.81±11.32 years), 3,936 belonged to patients with beta-thalassemia major, and 999 and 89 to patients with beta-thalassemia intermedia and sickle beta-thalassemia, respectively. In 467 cases, the type of thalassemia was not clear. The cumulative survival rate was calculated as 0.92, 0.83, 0.74, and 0.51 by ages 25, 35, 45, and 55, respectively. The hazard ratio of death was 4.22 (p<0.05) for beta-thalassemia major and 0.77 for beta-thalassemia intermedia (p=0.70). It was calculated as 1.45 for men patients and as 3.82 for single patients.CONCLUSIONSThe present study showed relatively high survival rates in patients with thalassemia. The survival of patients was unfavorable in poorer regions (Zahedan and Iranshahr). Factors including women gender, a higher level of education, being married, and living in metropolises decreased the risk of death at younger ages and improved survival.
Highlights
Beta-thalassemia is an inherited hemoglobin disorder in the beta-globin chain that results in chronic hemolytic anemia
The mean age of the thalassemia patients was estimated as 23.81 ± 11.32 years; it was very similar in Tehran and Sari (28.53 vs. 28.12 years), but was lower in Bandar-e-Abbas (Hormozgan) and Zahedan (18.92 and 15.88 years)
A significant difference was observed in the mean survival between men (51.47 years) and women (53.49 years), suggesting a longer survival in women, which is consistent with the results obtained in other studies; the mean survival reported for men and women in the present study is longer than has been reported in other studies [28]
Summary
Beta-thalassemia is an inherited hemoglobin disorder in the beta-globin chain that results in chronic hemolytic anemia. Two major forms of beta-thalassemia have been identified according to. In the long term, untreated TI causes gallstones, pulmonary hypertension, thrombophilia, osteoporosis, and foot ulcers [10,11,12]. These symptoms are rare in patients with TM. Untreated TI causes cardiac problems in the fourth decade of life through hypoxia and increased pulmonary resistance, potentially leading to death [1,11,13,14,15]
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
