Response of Patients with Intermediate Beta-Thalassemia to Treatment with Hydroxyurea

Document Type : Original Article (s)

Authors

1 Associate Professor, Department of Biology, School of Sciences, University of Isfahan, Isfahan, Iran

2 MSc Student, Department of Biology, School of Sciences, University of Isfahan, Isfahan, Iran

3 Assistant Professor, Department of Oncology and Radiotherapy, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran

4 General Practitioner, Inherited Blood Disorders Clinic, Sayed-al-Shohada Hospital, Isfahan, Iran

Abstract

Background: Hydroxyurea is a chemotherapeutic agent for treatment of cancer. This drug induces globin-g synthesis, so it could be used for treatment of thalassemia. Several studies have been shown that treatment with hydroxyurea increases Hb and HbF levels in patients with intermediate beta-thalassemia. However, the efficiency of hydroxyurea treatment in patients with beta-thalassemia is unclear. In the present study, clinical response of these patients to the drug was investigated.Methods: In this prospective study, the samples were patients with beta-thalassemia intermedia admitted to Sayed-al-Shohada hospital, Isfahan, Iran, during the years 2011-13. Efficiency of hydroxyurea in 46 patients was studied by determining the changes of Hb and HbF levels before and after one year of treatment with the drug. Treatment was performed using 500 mg capsule with dosage of 20 mg/day/kg. Patients were monitored for side effects, too.Findings: After treatment, the means of Hb and HbF levels increased at a rate of 0.47 ± 1.12 g/dl and 6.04 ± 1.43 percent, respectively; where the first was statistically significant, but the latter was not. Use of drug improved the quality of the patient's condition and there was no side effect.Conclusion: According to our results, it is suggested that treatment with hydroxyurea could be effective in majority of patients with intermediate beta-thalassemia.

Keywords

References

  1. Rund D, Rachmilewitz E. Beta-thalassemia. N Engl J Med 2005; 353(11): 1135-46.
  2. Perrine SP. Fetal globin induction--can it cure beta thalassemia? Hematology Am Soc Hematol Educ Program 2005; 38-44.
  3. Yuan J, Kannan R, Shinar E, Rachmilewitz EA, Low PS. Isolation, characterization, and immunoprecipitation studies of immune complexes from membranes of beta-thalassemic erythrocytes. Blood 1992; 79(11): 3007-13.
  4. Galanello R, Origa R. Beta-thalassemia. Orphanet J Rare Dis 2010; 5: 11.
  5. Goodman LS, Gilmam A. Goodman and Gilman's the pharmacological basis of therapeutics. 8th ed. New York, NY: Pergamon Press; 1990.
  6. 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(2): 652-6.
  7. Arruda VR, Lima CS, Saad ST, Costa FF. Successful use of hydroxyurea in beta-thalassemia major. N Engl J Med 1997; 336(13): 964.
  8. Cokic VP, Andric SA, Stojilkovic SS, Noguchi CT, Schechter AN. Hydroxyurea nitrosylates and activates soluble guanylyl cyclase in human erythroid cells. Blood 2008; 111(3): 1117-23.
  9. Tang DC, Zhu J, Liu W, Chin K, Sun J, Chen L, et al. The hydroxyurea-induced small GTP-binding protein SAR modulates gamma-globin gene expression in human erythroid cells. Blood 2005; 106(9): 3256-63.
  10. Choudhry VP, Lal A, Pati HP, Arya LS. Hematological responses to hydroxyurea therapy in multitransfused thalassemic children. Indian J Pediatr 1997; 64(3): 395-8.
  11. Loukopoulos D, Voskaridou E, Stamoulakatou A, Papassotiriou Y, Kalotychou V, Loutradi A, et al. Hydroxyurea therapy in thalassemia. Ann N Y Acad Sci 1998; 850: 120-8.
  12. Bradai M, Abad MT, Pissard S, Lamraoui F, Skopinski L, de Montalembert M. Hydroxyurea can eliminate transfusion requirements in children with severe beta-thalassemia. Blood 2003; 102(4): 1529-30.
  13. Italia KY, Jijina FJ, Merchant R, Panjwani S, Nadkarni AH, Sawant PM, et al. Response to hydroxyurea in beta thalassemia major and intermedia: experience in western India. Clin Chim Acta 2009; 407(1-2): 10-5.
  14. Ansari SH, Shamsi TS, Munzir S, Khan MT, Erum S, Perveen K, et al. Ggamma-Xmn I polymorphism: a significant determinant of beta-thalassemia treatment without blood transfusion. J Pediatr Hematol Oncol 2013; 35(4): e153-e156.
  15. Yavarian M, Karimi M, Bakker E, Harteveld CL, Giordano PC. Response to hydroxyurea treatment in Iranian transfusion-dependent beta-thalassemia patients. Haematologica 2004; 89(10): 1172-8.
  16. Hashemi A, Abrishamkar M, Jenabzade AR, Eslami Z. Hydroxyurea can reduce or eliminate transfusion requirements in children with major and intermediate thalassemia. Iran J Blood Cancer 2009; 1(4): 147-50.
  17. Ehsani MA, Hedayati-Asl AA, Bagheri A, Zeinali S, Rashidi A. Hydroxyurea-induced hematological response in transfusion-independent beta-thalassemia intermedia: case series and review of literature. Pediatr Hematol Oncol 2009; 26(8): 560-5.
  18. Karimi M, Haghpanah S, Farhadi A, Yavarian M. Genotype-phenotype relationship of patients with beta-thalassemia taking hydroxyurea: a 13-year experience in Iran. Int J Hematol 2012; 95(1): 51-6.
Journal of Isfahan Medical School
Volume 31, Issue 264 - Serial Number 264
November and December 2014
Pages 2023-2030

Files

History

  • Receive Date: 09 July 2013
  • Revise Date: 02 November 2024
  • Accept Date: 06 April 2022

Share

How to cite

Statistics