Vascular dysfunction in patients with young β-thalassemia: relation to cardiovascular complications and subclinical atherosclerosis

• Published in: Clinical and applied thrombosis/hemostasis Vol. 21; no. 8; p. 733
• Main Authors: Adly, Amira Abdel Moneam, El-Sherif, Nayera Hazaa, Ismail, Eman Abdel Rahman, El-Zaher, Yosra Abd, Farouk, Amal, El-Refaey, Asmaa Mohamed, Wahba, Mohammed Samy
• Format: Journal Article
• Language: English
• Published: United States 01-11-2015
• Subjects: Adolescent; Antigens, CD - blood; Atherosclerosis - blood; Atherosclerosis - etiology; Atherosclerosis - pathology; beta-Thalassemia - blood; beta-Thalassemia - complications; beta-Thalassemia - pathology; Cadherins - blood; Carotid Intima-Media Thickness; Cell-Derived Microparticles - metabolism; Cell-Derived Microparticles - pathology; Child; Endothelial Cells - metabolism; Endothelial Cells - pathology; Female; Humans; Male; Stem Cells - metabolism; Stem Cells - pathology; endothelial microparticles; von Willebrand factor antigen; thalassemia; vascular dysfunction; endothelial progenitor cells; erythropoietin
• ISSN: 1938-2723
• DOI: 10.1177/1076029614541515

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.