Impact of hydroxyurea on clinical and biological parameters of sickle cell anemia in children in Abidjan

Background: The lives of individuals affected by sickle cell disease are marked by painful crises sometimes accompanied by complications. Curative treatments such as bone marrow transplantation or gene therapy exist, but are not currently performed in Côte d'Ivoire. Treatment with hydroxyurea remains an effective alternative. The aim of our study is to contribute to improving the management of children with sickle cell disease.

Methods: We conducted a prospective observational study from November 2017 to April 2019 at the at the Yopougon University Hospital. Children aged 5 to 15 years experiencing at least 3 vaso-occlusive crises (VOC) per year were included in the study after obtaining informed and written consent from their parents. Each patient received a daily dose of 15mg/kg of hydroxyurea.

Results: The mean age of the children was 9 years. More than 75% of patients were  homozygous SSFA2 major sickle cell individuals. After 6 months on hydroxyurea, our study observed rates of 84.4%, 100%, and 97.8%, respectively, for the absence of vaso-occlusive crises, hospitalization, and transfusion. Biologically, from M0 to M12 the mean hemoglobin level increased significantly, from 7.24 to 8.55 g/dL; white blood cell (WBC) and platelet counts decreased; Fetal hemoglobin (Hb F) increased significantly from 10.3% to 19.7%. Biochemical parameters within normal ranges, except for a moderate treatment-related increase in transaminases.

Conclusion: The induction of fetal hemoglobin (Hb F) production through hydroxyurea intake is the primary mechanism by which hydroxyurea modifies the pathogenesis of sickle cell disease