Increased circulating activated endothelial cells, vascular endothelial growth factor, and tumor necrosis factor in thalassemia

• Published in: American journal of hematology 2002-06, Vol.70 (2), p.100-106
• Main Authors: Butthep, Punnee, Rummavas, Saknarong, Wisedpanichkij, Raewadee, Jindadamrongwech, Sumalee, Fucharoen, Suthat, Bunyaratvej, Ahnond
• Format: Article
• Language: English
• Subjects: Adult; Anemias. Hemoglobinopathies; Biological and medical sciences; Biomarkers; circulating endothelial cell; Diseases of red blood cells; Endothelial Growth Factors - blood; Endothelium, Vascular - cytology; Hematologic and hematopoietic diseases; Hemoglobin E - analysis; Hemoglobin H - analysis; Heterozygote; Humans; Lymphokines - blood; Medical sciences; Protein C - analysis; Protein S - analysis; Splenectomy; Surface Properties; thalassemia; Thalassemia - blood; Thalassemia - physiopathology; Tumor Necrosis Factor-alpha - analysis; Vascular Endothelial Growth Factor A; Vascular Endothelial Growth Factors; vascular perturbation
• ISSN: 0361-8609; 1096-8652
• DOI: 10.1002/ajh.10101

An increased number of circulating endothelial cells (CECs) was demonstrated in α‐ and β‐thalassemic patients, β‐thalassemia/hemoglobin E (BE), both splenectomized (BE[S]) and non‐splenectomized (BE[NS]), had higher numbers of CECs than α‐thalassemia, both HbH (α‐thal l/α‐thal 2; H) and HbH with hemoglobin Constant Spring (α‐thal 1/CS; H/CS). CECs were also increased in heterozygous HbE (EA) and homozygous HbE (EE). The highest level of tumor necrosis factor‐α (TNF‐α) was found in HbH/CS patients, whereas the highest levels of vascular endothelial growth factor (VEGF) was observed in BE[S] patients. Significant decreases, in protein C and protein S levels were found in both α‐ and β‐thalassemia compared with normal. Good correlations between the numbers of CEC and TNF‐α, VEGF, protein C, and protein S levels were demonstrated in this study. In addition, markers for endothelial cell activation and injury (intercellular adhesion molecule‐1, ICAM‐1/CD54; vascular cell adhesion molecule‐1, VCAM‐1/CD106; and E‐selectin, ELAM‐1/CD62E) were detected on the surface of isolated CECs using immunofluorescence technique. Appearance of CECs with markers for endothelial cell activation, together with increased levels of TNF‐α and VEGF and decreased levels of protein C and protein S in the circulation, may account for the propensity of vascular perturbation in thalassemic subjects. Am. J. Hematol. 70:100–106, 2002. © 2002 Wiley‐Liss, Inc.