Levels of soluble adhesion molecule vcam-1 and icam-1 in the plasma of chronic transfused children with sickle cell disease

Increased adhesive events between the blood vessel endothelium and red and white cells play a central role in the initiation of vasoocclusive crisis. We examined endothelial functions and serum levels of inflammatory mediators in transfusion-dependent patients with sickle cell disease in stable asymptomatic stage and on crisis. The study population consisted of 40 patients with sickle cell disease [SCD] aged from 5 years to 13 years and 35 healthy normal individual with matched age and sex as control group .Serum levels of vascular cell adhesive molecules [sVCAM-1] and intercellular adhesive molecule [sICAM-1] with serum level of interleukin 6 [IL6] were determined with Enzyme -Link Immunosorbent assay [ELISA]. Asymptomatic patients with sickle cell diseases had higher sVCAM-1 levels compared to normal controls [p<0.001]. Their levels were further elevated during acute episodes. Levels were significantly lower in chronic transfused child patients compared to acute crisis p= [<0.05].The reduction of adhesive molecule levels observed in our transfused SCD patients offers insight into the mechanism of the protective effect of transfusion against acute crisis especially painful and anemic crisis. SVCAM-1 and sICAM-1 levels inversely correlated with fetal hemoglobin in SCD patients. In conclusion, adhesive molecule expression uad cytokine production may also play a role in the disease outcome

Leukocytosis with HIV infection in a Saudi Arabian patient with sickle cell anemia

Transfusion associated Acquired Immunodeficiency Syndrome [AIDS] has recently been reported from Saudi Arabia. AIDS has been reported in a few sickle cell patients, most of whom have been heavily transfused. Patients with AIDS and AIDS related complex are usually neutropenic. This report describes a Saudi patient with sickle cell anaemia and transfusion acquired HIV disease who demonstrated a marked leukemoid reaction