Correlation Between Mean Red Cell Transfusion Demand And Chelation Therapy In Multiply Transfused Thalassemia Major Patients
Correlation Between Mean Red Cell Transfusion Demand And Chelation Therapy
Keywords:
Red Cell, Chelation Therapy, ThalassemiaAbstract
Background & Objective: Life long red blood transfusion remains the main treatment for β thalassemia major patients. Transfusion-dependent patients, in the absence of chelation therapy, develop progressive accumulation of iron, which is responsible for tissue damage and, eventually, death. The factors, which influence the iron burden are type of chelation therapy and mean red cell transfusion requirement. Increasing red cell transfusion requirement, iron deposit and development of all antibodies complicates transfusion therapy in thalassemia patients. Aim is to investigate the patients for the red cell transfusion requirement compared on the basis of iron overload and type of chelation therapy. Methodology: Ninety eights patients were included in this study and samples collected and investigated for the red cell transfusion requirement, compared on the basis of iron overload and type of chelation therapy.Conclusion: Combination of two iron chelators (such as parenteral desferroxamine plus oral deferiprone) have been shown to produce additive and synergistic effects, may produce enhanced iron excretion, minimize side effects, decrease mean red cell transfusion requirement and improve compliance is strongly recommended in transfusion dependent thalassemia patients.[Jain R NJIRM 2015; 6(4): 54-57]
References
2. Cario, H., Stahnke, K., Sander, S., Kohne, E. Epidemiological situation and treatment of patients with thalassemia major in Germany: results of the German multicenter β-thalassemia study. Annals of Hematology, 2000. 79, 7-12.
3. Cazzola, M., Borgna-Pignatti, C., Locatelli, F., Ponchio, L., Beguin, Y., De Stefano, P. A moderate transfusion regimen may reduce iron loading in beta-thalassemia major without producing excessive expansion of erythropoiesis. Transfusion, 1997. 37, 135-140.
4. Cazzola, M., De Stafano, P., Ponchio, L., Locatelli, F., Beguin, Y., Dessi, C., Barella, S., Cao, A., Galanello, R. Relationship between transfusion regime and suppression of erythropoiesis in beta-thalassemia major. British Journal of Hematology, 1995. 89, 473-478.
5. Brittenham, G.M., Griffith, P.M., Nienhuis, A.W., McClaren, C.E., Young, N.S.,Tucker, E.E., Allen, C.J., Farrell, D.E., Harris, J.W. Efficacy of deferoxamine in preventing complications of iron overload in patients with thalassemia major. New England Journal of Medicine, 1994. 331(9), 567-573.
6. Modell, B. and Berdoukas, V. The clinical approach to thalassemia. Grune and Stratton, New York and London, 1984. 332-397.
7. Olivieri, N.F., Nathan, D.G., MacMillan, J.H., Wayne, A.S., Liu, P.P., McGee, A., Martin, M., Koren, G., Cohen, A.R. Survival in medically treated patients with homozygous beta thalassaemia. New England Journal of Medicine, 1994. 331(9), 574-578.
8. Olivieri, N.F. and Brittenham, G.M. Iron-chelating therapy and the treatment of thalassemia. Blood, 1997. 89, 739-761.
9. Kontoghiorghes, G.J., Pattichi, K., Hadjigavriel, M., Kolnagou, A. Transfusional iron overload and chelation therapy with deferoxamine and deferiprone (L1). Transfusion Science, 2000. 23(3), 211-23.
10. Taher, A., Chamoun, F.M., Koussa, S., Saad, M.A., Khoriaty, A.I. Neeman, R., Mourad, F.H. Efficacy and side effects of deferiprone (L1) in thalassemia patients not compliant with desferrioxamine. Acta Haematologia, 1999. 101(4), 173-177.
