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Indian Journal of Hematology and Blood Transfusion
Published in: Indian Journal of Hematology and Blood Transfusion 2/2014

01-06-2014 | Review Article

Hydroxyurea in Sickle Cell Disease: Drug Review

Authors: Rohit Kumar Agrawal, Rakesh Kantilal Patel, Varsha shah, Lalit Nainiwal, Bhadra Trivedi

Published in: Indian Journal of Hematology and Blood Transfusion | Issue 2/2014

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Abstract

Hydroxyurea, a myelosuppressive agent, is the only effective drug proven to reduce the frequency of painful episodes. It raises the level of HbF and the haemoglobin level. It usually decreases the rate of painful episodes by 50 %. It was first tested in sickle cell disease in 1984. It also decreases the rate of ACS episodes and blood transfusions by ~50 % in adults. It was developed as an anticancer drug and has been used to treat myeloproliferative syndromes-leukemia, melanoma, and ovarian cancer. It was approved for use by FDA in adults. Side effects includes neutropenia, bone marrow suppression, elevation of hepatic enzymes, anorexia, nausea, vomiting and infertility.
Literature
1.
Brawley OW, Cornelius LJ, Edwards LR et al (2008) National Institutes of Health Consensus Development Conference statement: hydroxyurea treatment for sickle cell disease. Ann Intern Med 148(12):932–938PubMedCrossRef
2.
Leikin SL, Gallagher D, Kinney TR et al (1989) Mortality in children and adolescents with sickle cell disease: cooperative study of sickle cell disease. Pediatrics 84(3):500–508PubMed
3.
Platt OS, Brambilla DJ, Rosse WF et al (1994) Mortality in sickle cell disease: life expectancy and risk factors for early death. N Engl J Med 330(23):1639–1644PubMedCrossRef
4.
Odie`vre MH, Bony V, Benkerrou M et al (2008) Modulation of erythroid adhesion receptor expression by hydroxyurea in children with sickle cell disease. Haematologica 93(4):502–510CrossRef
5.
Gladwin MT, Shelhamer JH, Ognibene FP et al (2002) Nitric oxide donor properties of hydroxyurea in patients with sickle cell disease. Br J Haematol 116(2):436–444PubMedCrossRef
6.
Trompeter S, Roberts I (2009) Haemoglobin F modulation in childhood sickle cell disease. Br J Haematol 144(3):308–316PubMedCrossRef
7.
8.
Kato GJ, McGowan V, Machado RF et al (2006) Lactate dehydrogenase as a biomarker of hemolysis associated nitric oxide resistance, priapism, leg ulceration, pulmonary hypertension, and death in patients with sickle cell disease. Blood 107(2):2279–2285PubMedCentralPubMedCrossRef
9.
Charache S, Dover GJ, Moore RD et al (1992) Hydroxyurea: effects on hemoglobin F production in patients with sickle cell anemia. Blood 79(10):2555–2565PubMed
10.
Jayabose S, Tugal O, Sandoval C et al (1996) Clinical and hematologic effects of hydroxyurea in children with sickle cell anemia. J Pediatr 129(4):559–565PubMedCrossRef
11.
de Montalembert M, Belloy M, Bernaudin F et al (1997) Three-year follow-up of hydroxyurea treatment in severely ill children with sickle cell disease: the French Study Group on sickle cell disease. J Pediatr Hematol Oncol 19(4):313–318PubMedCrossRef
12.
Kinney TR, Helms RW, O’Branski EE et al (1999) Safety of hydroxyurea in children with sickle cell anemia: results of the HUG-KIDS study, a phase I/II trial. Pediatric Hydroxyurea Group. Blood 94(5):1550–1554PubMed
13.
Wang WC, Wynn LW, Rogers ZR, Scott JP, Lane PA, Ware RE (2001) A two-year pilot trial of hydroxyurea in very young children with sickle-cell anemia. J Pediatr 139(6):790–796PubMedCrossRef
14.
Ware RE, Eggleston B, Redding-Lallinger R et al (2002) Predictors of fetal hemoglobin response in children with sickle cell anemia receiving hydroxyurea therapy. Blood 99(1):10–14PubMedCrossRef
15.
Wang WC, Helms RW, Lynn HS et al (2002) Effect of hydroxyurea on growth in children with sickle cell anemia: results of the HUG-KIDS study. J Pediatr 140(2):225–229PubMedCrossRef
16.
de Montalembert M, Brousse V, Elie C et al (2006) Long-term hydroxyurea treatment in children with sickle cell disease: tolerance and clinical outcomes. Haematologica 91(1):125–128PubMed
17.
Hankins JS, Ware RE, Rogers ZR et al (2005) Longterm hydroxyurea therapy for infants with sickle cell anemia: the HUSOFT extension study. Blood 106(7):2269–2275PubMedCentralPubMedCrossRef
18.
Thornburg CD, Dixon N, Burgett S et al (2008) A pilot study of hydroxyurea to prevent chronic organ damage in young children with sickle cell anemia. Pediatr Blood Cancer 52(5):609–615CrossRef
19.
Zimmerman SA, Schultz WH, Burgett S, Mortier NA, Ware RE (2007) Hydroxyurea therapy lowers transcranial Doppler flow velocities in children with sickle cell anemia. Blood 110(3):1043–1047PubMedCrossRef
20.
Ware RE, Zimmerman SA, Schultz WH (1999) Hydroxyurea as an alternative to blood transfusions for the prevention of recurrent stroke in children with sickle cell disease. Blood 94(9):3022–3026PubMed
21.
Ryan K, Heeney M (2004) New England pediatric sickle cell consortium, 13 May 2004
22.
Heeney MM, Whorton MR, Howard TA, Ware RE (2004) Chemical and functional analysis of hydroxyurea oral solutions. J Pediatr Hematol Oncol 26(3):179–184PubMedCrossRef
23.
Heeney MM, Ware RE (2008) Hydroxyurea for children with sickle cell disease. Pediatr Clin North Am 55(2):483–501PubMedCrossRef
24.
Ballas SK, McCarthy WF, Guo N et al (2009) Multicenter study of hydroxyurea in sickle cell anemia. Exposure to hydroxyurea and pregnancy outcomes in patients with sickle cell anemia. J Natl Med Assoc 101:1046–1051PubMed
Metadata
Title
Hydroxyurea in Sickle Cell Disease: Drug Review
Authors
Rohit Kumar Agrawal
Rakesh Kantilal Patel
Varsha shah
Lalit Nainiwal
Bhadra Trivedi
Publication date
01-06-2014
Publisher
Springer India
Published in
Indian Journal of Hematology and Blood Transfusion / Issue 2/2014
Print ISSN: 0971-4502
Electronic ISSN: 0974-0449
DOI
https://doi.org/10.1007/s12288-013-0261-4

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