Demographic and clinical profiles of patients with β-thalassemia major treated at Dubai Thalassemia Centre: A retrospective study

Abstract

Background: β-thalassemia major (BTM) is one of the hereditary anemias that is inherited as autosomal recessive. It is complicated by iron overload in different organs including the heart, liver, and endocrine glands. This study aimed to look at the demographic of patients with BTM treated at Dubai Thalassemia Centre. Methods: Demography and clinical data for all thalassemia patients between 2 and 45 years of age were collected. Data such as age, gender, nationality, frequency of blood transfusion, and ferritin level among different groups were calculated. Results: A total of 351 patients were studied; men constituted 50.7% (n = 178) compared with women 49.3% (n = 173). Young children (2–9 years) made up 11.1% (n = 39) of the total population studied, whereas older children and adolescents (10–18 years) made 20.2% (n = 71), and adults (19–45 years) made 67.7% (241). UAE nationals constituted 38% of the total population compared with 44% in previously published studies. Also, the percentage of Emirati children has dropped significantly from prior years (46.3% to 19.9%). The mean pre-transfusion hemoglobin ranged between 9.69–10.2 g/dL in all age groups indicating a successful hyper-transfusion policy. The median Ferritin level was significantly higher among women aged 19–45 years (3255 µg/L) as compared with men (2160 µg/L; U = 5488, P = 0.034) in the same group, and this was not observed in the other groups. Conclusion: The success of the premarital screening program implemented by the UAE government was evident in the drop in the percentage of Emirati children affected by BTM in our study.

Keywords

• demography
• ferritin
• pre-transfusion hemoglobin
• thalassemia