ISSN 2997-1036
International Journal of Hematology | Vol. 13, No. 2, February 2022 | pp. 9–16
DOI: 10.46882/2022/IJH/000144
Original Article
Title: Impact of systemic hydroxyurea on plasma soluble intercellular adhesion molecule-1 levels and painful crisis patterns in pediatric sickle cell anemia
Names of Authors: S. T. Adeyemi¹, U. V. Okoye², W. X. Salami³
Authors’ Affiliations: ¹Department of Haematology and Blood Transfusion, Obafemi Awolowo University, Ile-Ife, Nigeria; ²Department of Medicine, University of Nigeria Teaching Hospital, Enugu, Nigeria; ³Department of Paediatrics, Ahmadu Bello University, Zaria, Nigeria
Abstract: Microvascular occlusion in sickle cell disease is driven by complex adhesive interactions between sickle erythrocytes, leukocytes, and the vascular wall. This prospective cohort study evaluated the long-term impact of optimized hydroxyurea therapy on plasma soluble intercellular adhesion molecule-1 (sICAM-1) concentrations and clinical pain crisis patterns in 54 pediatric patients (aged 3 to 12 years) with steady-state sickle cell anemia (HbSS). Hydroxyurea was administered at a mean dose of 20 mg/kg/day and monitored over 12 months. Plasma markers were quantified using an enzyme-linked immunosorbent assay at baseline, 6 months, and 12 months. Baseline mean sICAM-1 concentrations fell significantly from 342.5 ± 48.0 ng/ml to 184.2 ± 22.0 ng/ml at month 12 (P < 0.001). Concurrently, the annual frequency of documented vaso-occlusive painful crises fell by 72.4%. Reductions in sICAM-1 values correlated positively with an increase in fetal hemoglobin from 5.2% to 16.5% and a drop in absolute reticulocyte counts. These findings confirm that hydroxyurea successfully downregulates endothelial adhesion molecules, providing a reliable and non-invasive pharmacological pathway for reducing microvascular cellular entrapment in pediatric sickle cell variants.
Keywords: Sickle cell anemia, hydroxyurea, ICAM-1, cellular adhesion, pediatric vaso-occlusion
Manuscript Timeline: Received: November 05, 2021; Revised: December 14, 2021; Accepted: January 08, 2022; Published: February 18, 2022