Abstract
Fifteen multi-transfused thalassemic children between 3–6 years of age (seven boys and eight girls) were administered hydroxyurea for 5 days and three such cycles were repeated at 15 days interval. Rise in hemoglobin levels was observed in 13, 8 and 7 cases, while rise in mean hemoglobin F was seen in 8, 11 and 10 cases following 1st, 2nd and 3rd course of hydroxyurea therapy respectively. Rise in hemoglobin level was associated with transient rise in fetal hemoglobin level. Though the changes in mean hemoglobin levels and hemoglobin F levels were statistically insignificant, a definite trend of rise in hemoglobin and hemoglobin F was observed.
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References
Wood WG, Weatherall DJ. Developmental genetics of the human hemoglobins.Biochem J 1983; 215: 1–10.
Karlsson S, Nienhuis AW. Developmental regulation of human globin genes.Annu Rev Biochem 1985; 54: 1071–1108
LuCarelli G, Angelucci E, Giardini C, Baronciani D, Galimberts M, Palchi P and Erer B. Bone marrow transplantation in thalassemia.Ind J Pediatr 1993; 60: 517–523.
Gallo E, Massaro P, Miniero R, David D, Tarella C. The importance of the genetic picture and globin synthesis in determining the clinical and hematological features of thalassemia intermedia.Br J Hematol 1979; 41: 211–221.
Ley TJ, DeSimone J, Anagnou NP,et al. 5-Azacytidine selectively increases Y-globin synthesis in a patient with β-thalassemia.N Engl J Med 1982; 307: 1469–1475.
Platt OS, Orkin SH, Dover G, Beardsley GP, Miller B, Nathan DG. Hydroxyurea enhances fetal hemoglobin production in sickle cell anemia.J Clin invest 1984; 74: 652–656.
Charalhe S, Dover DG, Moyer MA, Moore JW. Hydroxyurea induced augmentation of fetal hemoglobin production in patients with sicle cell anemia.Blood 1987; 69: 109–116.
Rodgers GP, Dover GJ, Noguchi CT, Schechter AN, Niehuis AW. Hematologic responses of patients with sickle cell disease to treatment with Hydroxyurea.N Eng J Med 1980; 322: 1037–1045.
Perrine SP, Ginder GD, Faller DV,et al. A short term trial of butyrate to stimulate fetal globin gene expression in the beta globin disorders.N Eng J Med 1993; 328: 81–86.
Cleg JB, Weatherhall D, Bodmer WF. 5-Azacytidine for beta thalassemia.Lancet 1983; 1: 536–541.
Platt OS, Orkin SH, Dover G, Beardsley P, Murlee B and Nathan DJ. Hydroxyurea enhances Hb F production in sickle cell anemia.J Clin Invest 1984; 74: 652–656.
Dover GJ, charache S Smith KD: Hydroxyurea induced augmentation of fetal hemoglobin production in patients with sickle cell anemia.Blood 1987; 69: 109–116.
Busslinger M, Hurst J, Fraveil RA: DNA methylation and the regulation of globin gene expression.Cell 1983; 34: 197–206.
Fucharoen S, Siritanaratbul N, Winichagoon P,et al. Hydroxyurea increases Hb F levels and improves the effectiveness of erythropoiesis in β thalassemia/Hb E disease.Inter Conf. thalassemia, Bombay, November 1993; 56 (Abstract).
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Choudhry, V.P., Lal, A., Pati, H.P. et al. Hematological responses to hydroxyurea therapy in multitransfused thalassemic children. Indian J Pediatr 64, 395–398 (1997). https://doi.org/10.1007/BF02845212
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DOI: https://doi.org/10.1007/BF02845212