Commentary

Video

Achieving Quick Responses in Sickle Cell Anemia With Early, Appropriate Hydroxyurea Dosing, with Abena Appiah-Kubi, MD, MPH

Appiah-Kubi also noted that despite elevated HbF levels, very young children with SCA already had anemia and reticulocytosis.

Early initiation and appropriate titration of hydroxyurea led to quick fetal hemoglobin (HbF) responses in children with sickle cell anemia (SCA).

These findings, from the Hydroxyurea Optimization through Precision Study (HOPS, NCT03789591), were presented at the 66th American Society of Hematology (ASH) Annual Meeting and Exposition, held December 7-10, 2024, in San Diego, California, by Abena Appiah-Kubi, MD, MPD, Associate Professor of pediatrics at Zucker School of Medicine at Hofstra/Northwell.

Appiah-Kubi and colleagues found that overall, early initiation of hydroxyurea with dose escalation resulted in significant hematologic improvement, with increases in hemoglobin from 9.0 g/dL (standard deviation [SD], 1.1) to 10.1 (SD, 1.4; P <.001), Absolute Reticulocyte Count from 265 (SD, 123) to 185 (SD, 110; P <.001), and HbF from 27.4% (SD, 11.2) to 34.3% (SD, 10.3; P <.001). Most (74%) participants achieved HbF levels of at least 30% and 31% had HbF levels of at least 40% at month 6, compared to 46% and 13%, before treatment initiation.

“We recommend that we can start children with SCA on hydroxyurea even at a young age, despite whatever their HbF levels are, because… in these very young children, they actually already had anemia and reticulocytosis, so they were already showing us evidence of hemolysis with the illness, even at that very young age. Their HbF levels were around 27% in both arms and despite that, we still had these findings. And so don't be afraid to start it early. Even if the HbF level is elevated, we see remarkable improvement in all hematologic markers,” Appiah-Kubi told HCPLive® during the meeting.

REFERENCE
Appiah-Kubi A, Jacob SA, Heeney MM, et al. Individualized, PK-Guided Dosing of Hydroxyurea for Young Children with Sickle Cell Anemia: Final Results from the Hydroxyurea Optimization through Precision Study (HOPS). Presented at: ASH Annual meeting; December 7-10; San Diego, California. Abstract 175.
Related Videos
Caroline Piatek, MD: High HCRU, Patient Concerns Highlight Great Unmet Need in wAIHA
4 experts are featured in this series.
Steven W. Pipe, MD: Supporting Gene Therapy Implementation for Hemophilia
Corinna L. Schultz, MD: Improving Sickle Cell Trait Documentation in Infancy
Skin of Color Savvy: The Art and Science of Treating Patients of Color
© 2025 MJH Life Sciences

All rights reserved.