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Blood, 15 March 2004, Vol. 103, No. 6, pp. 2039-2045. Prepublished online as a Blood First Edition Paper on November 20, 2003; DOI 10.1182/blood-2003-07-2475. This Article Full Text (PDF) All Versions of this Article: 2003-07-2475v1 103/6/2039    most recent Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Zimmerman, S. A Articles by Ware, R. E Search for Related Content PubMed PubMed Citation Articles by Zimmerman, S. A Articles by Ware, R. E Social Bookmarking                   What's this? Submitted July 24, 2003 Accepted November 11, 2003 Sustained long-term hematological efficacy of hydroxyurea at maximal tolerated dose in children with sickle cell disease Sherri A Zimmerman*, William H Schultz, Jacqueline S Davis, Chrisley V Pickens, Nicole A Mortier, Thad A Howard, and Russell E Ware Department of Pediatrics, Duke University Medical Center, Durham, NC, USA * Corresponding author; email: zimme008{at}mc.duke.edu. Hydroxyurea improves hematological parameters for children with sickle cell disease (SCD) but its long-term efficacy at maximal tolerated dose (MTD) has not been determined. Between 1995 and 2002, hydroxyurea therapy was initiated for 122 pediatric patients with SCD, including 106 with HbSS, 7 with HbSC, 7 with HbS/-thalassemia (6 HbS/°, 1 HbS/+), and 2 with HbS/OArab. Median age at initiation of therapy was 11.1 years. Hydroxyurea was escalated to MTD with an average dose of 25.4 ± 5.4 mg/kg/day; the average duration of hydroxyurea therapy has been 45 ± 24 months (range 6 to 101 months). Fifteen children (12%) with poor compliance had hydroxyurea discontinued. Mild transient neutropenia occurred but no hepatic or renal toxicity was noted. Hydroxyurea therapy led to significant increases in hemoglobin, mean corpuscular volume, and fetal hemoglobin (HbF), while significant decreases occurred in reticulocytes, WBC, platelets, and serum bilirubin. Children with variant SCD genotypes also had hematological responses to hydroxyurea therapy. HbF induction has been sustained for up to 8 years without adverse effects on growth or increased numbers of acquired DNA mutations. Long-term hydroxyurea therapy at MTD is well tolerated by pediatric patients with SCD and has sustained hematological efficacy with apparent long-term safety. 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