Vascular Dysfunction in Patients With Young β-Thalassemia

Abstract

We aimed to study the endothelial dysfunction among children and adolescents with transfusion-dependent β-thalassemia using von Willebrand factor antigen (VWF:Ag) and flow cytometric analysis of circulating CD144+ endothelial microparticles (EMPs) and endothelial progenitor cells (CD34+VEGFR2+) and assess their relation to iron overload, erythropoietin and chelation therapy as well as echocardiographic parameters and carotid intima–media thickness. The VWF:Ag, EMPs, and CD34+VEGFR2+ cells were significantly higher among patients with β-thalassemia than controls ( P < .001). The type of chelation and patients’ compliance did not influence the results. No significant correlations were found between the studied vascular markers. Patients with evident heart disease had higher VWF: Ag, EMPs, and CD34+VEGFR2+ cells than those without. Carotid intima–media thickness was increased among patients but not correlated with vascular markers. We suggest that procoagulant EMPs and VWF: Ag are involved in cardiovascular complications in patients with young β-thalassemia. CD34+VEGFR2+ cells were further increased in response to tissue injury contributing to reendothelialization and neovascularization.