Associate Professor of pediatrics at Zucker School of Medicine at Hofstra/Northwell.

Abena Appiah-Kubi, MD, MPH

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

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

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; 

<.001), Absolute Reticulocyte Count from 265 (SD, 123) to 185 (SD, 110; 

<.001), and HbF from 27.4% (SD, 11.2) to 34.3% (SD, 10.3; 

<.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 

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.

Abena Appiah-Kubi, MD, MPH

Articles