Prevalence of Duffy null and its impact on hydroxyurea in young children with sickle cell disease in the United States

Pediatr Blood Cancer. 2024 Jun;71(6):e30945. doi: 10.1002/pbc.30945. Epub 2024 Mar 10.

Abstract

Consistent with studies showing a high prevalence of the Duffy null phenotype among healthy Black Americans, this retrospective study found that Duffy null was present in >75% of a young and contemporary cohort of children with sickle cell disease (SCD) in the United States. Despite the potential for this phenotype to impact absolute neutrophil counts, hydroxyurea (HU) dosing, and outcomes, it was not associated with being prescribed a lower HU dose or having increased acute SCD visits early in the HU treatment course. Future studies are needed to confirm these findings in older children with SCD.

Keywords: hydroxyurea; outcomes research; sickle cell anemia; sickle cell disease.

Publication types

  • Research Support, N.I.H., Extramural

MeSH terms

  • Adolescent
  • Anemia, Sickle Cell* / drug therapy
  • Anemia, Sickle Cell* / epidemiology
  • Antisickling Agents* / therapeutic use
  • Child
  • Child, Preschool
  • Duffy Blood-Group System* / genetics
  • Female
  • Humans
  • Hydroxyurea* / administration & dosage
  • Hydroxyurea* / therapeutic use
  • Infant
  • Male
  • Prevalence
  • Receptors, Cell Surface / genetics
  • Retrospective Studies
  • United States / epidemiology

Substances

  • Hydroxyurea
  • Duffy Blood-Group System
  • Antisickling Agents
  • ACKR1 protein, human
  • Receptors, Cell Surface