Premature Atherosclerosis in Non-Transfusion-Dependent β-Thalassemia Intermedia

Abstract

Objectives: β-Thalassemias are inherited hemolytic anemias with a broad phenotype and an increased rate of vascular complications despite a near absence of coronary artery disease. Methods: We investigated the presence of endothelial dysfunction and subclinical atherosclerosis in non-transfusion-dependent patients with β-thalassemia intermedia (β-TI) by means of flow-mediated (FMD) and flow-independent (FID) brachial artery dilatation and carotid artery intima-media thickness. Results: In 20 nondiabetic young adults with β-TI, FMD (6.6 ± 3.7 vs. 10.3 ± 3.1%; p = 0.002) and FID (14.0 ± 4.7 vs. 18.0 ± 5.6%; p = 0.02) were both lower relative to the values in 20 matched control subjects, whereas the intima-media thickness was increased (0.51 ± 0.09 vs. 0.46 ± 0.07 mm; p = 0.049). Fibrin generation, soluble endothelial activation markers, and proinflammatory proteins were higher in the patient group, while the plasma cholesterol level was lower. Conclusions: These findings indicate premature atherosclerosis among patients with β-TI; this is in accord with the high incidence of noncoronary vascular episodes in β-TI.