Study of soluble vascular cell adhesion molecule one [sVCAM-1] and von will brand factor [vWF] in children with beta thalassemia major

ABSTRACT

There is a strong evidence of endothelial cell activation in beta thalassemia and vascular complications in these patients are more frequent. The objective of this work is to study the levels of circulating soluble vascular cell adhesion molecule [sVCAM-1] and Von Will brand factor [vWF] as parameters of endothelial cell activation in a group of children with P-thalassemia major, and their relation to duration of the disease. The study was conducted on 35 patients with P-thalassemia major, recruited from the hematology outpatient clinic of Alexandria University Children's Hospital, who were divided into two groups. Group I included 15 young children with a disease duration 55 years and group I1 included 20 older children and adolescents with disease duration > 5 years. Ten apparently healthy children of matching age and sex served as control group. Children were subjected to full clinical examination with special emphasis on signs of vascular affection. Serum levels of sVCAM-1 were measured by enzyme- linked immunosorbent assay [ELISA], and vWF by radial immunodiffusion. The results showed that 4 of the studied patients had pulmonary hypertension and one patient suffered from left lower limb deep vein thrombosis. Soluble vascular cell adhesion molecule-1 results demonstrated significantly higher levels in both thalassemic groups in comparison with controls and non-significant difference between the two groups themselves. A positive correlation was found between sVCAM-1 levels and serum ferritin in both groups, and with vWF in-group II only [P=0.007, 0.042 and 0.031 respectively]. There was a non-significant difference between the levels of vWF in both thalassemic groups and controls [k0.093]. There was no statistically significant difference as regards sVCAM-1 and vWF levels in non-splenectomized and splenectomized children in both groups [P=0.545, P=0.219]