Beta-Thalassämie major und damit verbundene Komplikationen

Beta-thalassemia major (TM) remains one of the largest health problems, particularly in developing countries. Tunisia is among the Mediterranean countries most affected by this disease, which primarily occurs in small towns among low-income families. The main objectives of this study are to describe the demographic and clinical characteristics as well as the transfusion-related complications in patients with TM in Tunisia. A standardized questionnaire was sent to clinicians in 33 different medical facilities that care for thalassemia patients. A total of 391 transfusion-dependent thalassemia patients with an average age of 10.7 years (ranging from 3 months to 31 years) were included in the study. Most of them were from the northwest region of the country. Moderate iron overload between 1501 and 2500 ng/ml was found in 61 patients, while 81 patients (26.9%) had a ferritin level of more than 2500 ng/ml and 21 patients (6.9%) had a level of more than 5000 ng/ml. Fifty-one patients died from complications related to their disease, with heart failure being the leading cause of death. The frequency of cardiac, endocrine, and infectious complications is being investigated.