title: Hydroxyurea decreases hospitalizations in pediatric patients with Hb SC and Hb SB+ thalassemia.
abstract: OBJECTIVE
  Patients with hemoglobin SC (Hb SC) and hemoglobin SB+ (Hb SB+) thalassemia suffer from frequent hospitalizations yet strong evidence of a clinical benefit of hydroxyurea (HU) in this population is lacking. Patients with recurrent hospitalizations for pain crisis are offered HU at our institution based on small cohort data and anecdotal benefit. This study identifies outcomes from a large cohort of patients with Hb SC and SB+ thalassemia who were treated with HU for 2 years.
  
  
  METHODS
  A retrospective review was conducted of 32 patients with Hb SC and SB+ thalassemia who were treated with HU. We reviewed the number, and reasons for hospitalization in the 2 years prior to, and 2 years post-HU treatment as well as laboratory changes from baseline, over 1 year.
  
  
  RESULTS
  Patients with Hb SC and SB+ thalassemia started on HU for frequent pain, had a significant reduction in hospitalizations over 2 years as compared to the 2 years prior to HU initiation (mean total hospitalizations/year: pre-HU: 1.6 vs post-HU 0.4 hospitalizations, P<0.001; mean pain hospitalizations/year: pre-HU 1.5 vs post-HU 0.3 hospitalizations, P<0.001). Patients demonstrated hematologic changes including an increase in percent fetal hemoglobin (%HbF) pre-post HU (4.5% to 7.7%, P=0.002), mean corpuscular volume (74 to 86 fL, P<0,0001), and decrease in absolute neutrophil count (5.0 to 3.2×10(9)/L, P=0.007). Patients with higher doses of HU demonstrated the greatest reduction in hospitalizations but this was unrelated to absolute neutrophil count.
  
  
  CONCLUSIONS
  This cohort of patients with Hb SC and SB+ thalassemia provides additional support for using HU in patients with recurrent hospitalizations for pain. A large randomized multicenter trial of HU to reduce pain admissions should be conducted to confirm these data and provide much needed evidence based recommendations for this population.
authors.affiliation: Division of Pediatric Hematology Oncology, University of Alabama at Birmingham, Birmingham, AL, USA.
authors.forename: Jeffrey D
authors.initials: JD
authors.lastname: Lebensburger
authors.affiliation: Division of Pediatric Hematology Oncology, University of Alabama at Birmingham, Birmingham, AL, USA.
authors.forename: Rakeshkumar J
authors.initials: RJ
authors.lastname: Patel
authors.affiliation: Division of Pediatric Hematology Oncology, University of Alabama at Birmingham, Birmingham, AL, USA.
authors.forename: Prasannalaxmi
authors.initials: P
authors.lastname: Palabindela
authors.affiliation: Division of Pediatric Hematology Oncology, University of Alabama at Birmingham, Birmingham, AL, USA.
authors.forename: Christina J
authors.initials: CJ
authors.lastname: Bemrich-Stolz
authors.affiliation: Division of Pediatric Hematology Oncology, University of Alabama at Birmingham, Birmingham, AL, USA.
authors.forename: Thomas H
authors.initials: TH
authors.lastname: Howard
authors.affiliation: Division of Pediatric Hematology Oncology, University of Alabama at Birmingham, Birmingham, AL, USA.
authors.forename: Lee M
authors.initials: LM
authors.lastname: Hilliard
keywords: hydroxyurea; outcomes research; sickle cell disease; vaso-occlusive pain crisis