International Journal of Hematology

ISSN 2997-1036

International Journal of Hematology | Vol. 7, No. 2, February 2016 | pp. 9–16
DOI: 10.46882/2016/IJH/000074

Original Article

Title: Impact of hydroxyurea optimization on transcranial Doppler velocity variations in children with 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: Elevated time-averaged maximum mean velocities on transcranial Doppler (TCD) ultrasonography identify children with sickle cell anemia who carry an exceptionally high risk of primary stroke. This prospective cohort study evaluated the efficacy of optimized hydroxyurea therapy for reducing elevated transcranial Doppler velocities in 54 pediatric patients (aged 2 to 12 years) presenting with conditional (170 to 199 cm/s) or abnormal (≥ 200 cm/s) baseline velocities. Hydroxyurea was initiated at 15 mg/kg/day and titrated to the maximum tolerated dose over 12 months. Repeat transcranial Doppler evaluations were performed at 6 and 12 months. The mean baseline velocity fell significantly from 186.4 ± 12.5 cm/s to 154.2 ± 10.4 cm/s at month 12 (P < 0.001). Concurrently, fetal hemoglobin concentrations rose from a baseline mean of 6.2 ± 1.8% to 16.5 ± 3.2%. None of the children in the conditional or abnormal cohorts progressed to a primary stroke during the study window. Optimizing hydroxyurea to maximum tolerated doses effectively reduces high cerebrovascular blood flow velocities, providing an accessible and non-invasive pharmacological pathway for primary stroke prevention in pediatric sickle cell populations.

Keywords: Sickle cell anemia, hydroxyurea, transcranial Doppler, fetal hemoglobin, pediatric stroke

Manuscript Timeline: Received: November 05, 2015; Revised: December 14, 2015; Accepted: January 08, 2016; Published: February 18, 2016