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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 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 Citation Map 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. Related Collections Red Cells Clinical Trials and Observations Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  CLINICAL OBSERVATIONS, INTERVENTIONS, AND THERAPEUTIC TRIALS Sustained long-term hematologic efficacy of hydroxyurea at maximum 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 From the Duke Pediatric Sickle Cell Program and Division of Pediatric Hematology/Oncology, Duke University Medical Center, Durham, NC. Hydroxyurea improves hematologic parameters for children with sickle cell disease (SCD), but its long-term efficacy at maximum tolerated dose (MTD) has not been determined. Between 1995 and 2002, hydroxyurea therapy was initiated for 122 pediatric patients with SCD including 106 with homozygous sickle cell anemia (HbSS), 7 with sickle hemoglobin C (HbSC), 7 with sickle/-thalassemia (HbS/ -thalassemia [6 HbS/0, 1 HbS/+]), and 2 with sickle hemoglobin OArab (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 per day; the average duration of hydroxyurea therapy has been 45 ± 24 months (range, 6-101 months). Hydroxyurea was discontinued for 15 (12%) children with poor compliance. Mild transient neutropenia occurred, but no hepatic or renal toxicity was noted. Hydroxyurea therapy led to significant increases in hemoglobin level, mean corpuscular volume, and fetal hemoglobin (HbF) level, whereas significant decreases occurred in reticulocyte, white blood cell, and platelet counts and serum bilirubin levels. Children with variant SCD genotypes also had hematologic responses to hydroxyurea. 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 hematologic efficacy with apparent long-term safety. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: S. Lanzkron, J. J. Strouse, R. Wilson, M. C. Beach, C. Haywood, H. Park, C. Witkop, E. B. Bass, and J. B. Segal Systematic Review: Hydroxyurea for the Treatment of Adults with Sickle Cell Disease Ann Intern Med, June 17, 2008; 148(12): 939 - 955. [Abstract] [Full Text] [PDF] R. E. Ware TCD response to hydroxyurea therapy Blood, January 15, 2008; 111(2): 964 - 964. [Full Text] [PDF] A. Marusyk, L. J. Wheeler, C. K. Mathews, and J. DeGregori p53 Mediates Senescence-Like Arrest Induced by Chronic Replicational Stress Mol. Cell. Biol., August 1, 2007; 27(15): 5336 - 5351. [Abstract] [Full Text] [PDF] S. A. Zimmerman, W. H. Schultz, S. Burgett, N. A. Mortier, and R. E. Ware Hydroxyurea therapy lowers transcranial Doppler flow velocities in children with sickle cell anemia Blood, August 1, 2007; 110(3): 1043 - 1047. [Abstract] [Full Text] [PDF] S. Austin, H. Cohen, and N. Losseff Haematology and neurology J. Neurol. Neurosurg. Psychiatry, April 1, 2007; 78(4): 334 - 341. [Abstract] [Full Text] [PDF] A. D. Villella Hydroxyurea Therapy for Infants with Sickle Cell Anemia AAP Grand Rounds, April 1, 2006; 15(4): 39 - 40. [Full Text] [PDF] H. Fathallah and G. F. Atweh Induction of Fetal Hemoglobin in the Treatment of Sickle Cell Disease Hematology, January 1, 2006; 2006(1): 58 - 62. [Abstract] [Full Text] [PDF] J. S. Hankins, R. E. Ware, Z. R. Rogers, L. W. Wynn, P. A. Lane, J. P. Scott, and W. C. Wang Long-term hydroxyurea therapy for infants with sickle cell anemia: the HUSOFT extension study Blood, October 1, 2005; 106(7): 2269 - 2275. [Abstract] [Full Text] [PDF] B. S. Coller Leukocytosis and Ischemic Vascular Disease Morbidity and Mortality: Is It Time to Intervene? Arterioscler. Thromb. Vasc. Biol., April 1, 2005; 25(4): 658 - 670. [Abstract] [Full Text] [PDF] B. Gulbis, D. Haberman, D. Dufour, C. Christophe, C. Vermylen, F. Kagambega, F. Corazza, C. Devalck, M.-F. Dresse, K. Hunninck, et al. Hydroxyurea for sickle cell disease in children and for prevention of cerebrovascular events: the Belgian experience Blood, April 1, 2005; 105(7): 2685 - 2690. [Abstract] [Full Text] [PDF] G. R. Buchanan, M. R. DeBaun, C. T. Quinn, and M. H. Steinberg Sickle Cell Disease Hematology, January 1, 2004; 2004(1): 35 - 47. [Abstract] [Full Text] [PDF] J. S. Hankins, R. E. Ware, Z. R. Rogers, L. W. Wynn, P. A. Lane, J. P. Scott, and W. C. 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