An age dependent response to hydroxyurea in pediatric sickle cell anemia patients with alpha thalassemia trait

Lisa Figueiredo, Kerry Morrone, Catherine Wei, Karen Ireland, Hillel W. Cohen, Catherine Driscoll, Deepa Manwani

Research output: Contribution to journalArticlepeer-review

Abstract

Hydroxyurea (HU) is a key drug therapy for individuals with sickle cell anemia (SCA), yet its clinical and hematologic responses can be variable. Various studies have reported the role of α-thalassemia as one of the most prevalent heritable traits that may modify HU response. We provide data from 62 pediatric and adolescent patients with SCA, 26 with co-inherited α-thalassemia trait. Our data suggest that altered hematologic and clinical responses to HU therapy are noted in adolescent SCA individuals with co-inherited α-thalassemia trait. Adolescent patients who co-inherited α-thalassemia trait had a greater reduction in vaso-occlusive episodes compared to those without α-thalassemia, despite a less robust fetal hemoglobin induction as well as a lower maximum HU dose. This clinical improvement was associated with a lower MCH and higher RBC count. Responses to HU in younger SCA children (ages 5–11 years) with co-inherited α-thalassemia trait, compared to those without α-thalassemia trait, did not show any difference in number vaso-occlusive episodes, fetal hemoglobin induction and change in MCH and RBC count.

Original languageEnglish (US)
Pages (from-to)19-23
Number of pages5
JournalBlood Cells, Molecules, and Diseases
Volume66
DOIs
StatePublished - Jul 2017

Keywords

  • Alpha thalassemia
  • Hydroxyurea
  • Sickle cell anemia

ASJC Scopus subject areas

  • Molecular Medicine
  • Molecular Biology
  • Hematology
  • Cell Biology

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