Transfusion therapy has revolutionized the care of patients with thalassaemia major, dramatically improving life expectancy when appropriately administered.
Regular transfusions improve survival in thalassaemia patients
Regular blood transfusions dramatically extend life expectancy in patients with thalassaemia major. Adapted with permission [1]
PRACTICAL STEPS FOR TRANSFUSING PATIENTS WITH THALASSAEMIA
Several steps can be taken to maximize the benefits of transfusion therapy in patients with thalassaemia:
Regular transfusions (every 2-4 weeks) are recommended [2].
Various regimens have been used, including hypertransfusion, supertransfusion, and moderate transfusion programs. The moderate regimen can ensure normal growth without excessive expansion of erythropoiesis, and with effective prevention of iron overload [3].
In patients whose yearly transfusion requirement exceeds 200 mL of packed red cells/kg body weight, appropriately timed splenectomy can significantly reduce transfusion requirements and iron accumulation [4;5]. Because of the risk of infection, it is generally avoided until at least the age of 4-5 years [6].
Iron chelation therapy may be indicated after 10-20 transfusions or when serum ferritin levels exceed 1000 ng/mL [6;7].
References (1) Gabutti V, Piga A: Results of long-term iron-chelating therapy. Acta Haematol 1996; 95(1):26-36.
(2) Wonke B: Clinical management of beta-thalassemia major. Semin Hematol 2001; 38(4):350-359.
(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(2):135-140.
(4) Cohen A, Gayer R, Mizanin J: Long-term effect of splenectomy on transfusion requirements in thalassaemia major. Am J Hematol 1989; 30(4):254-256.
(5) Modell B: Total management of thalassaemia major. Arch Dis Child 1977; 52(6):489-500.
(6) Fosburg MT, Nathan DG: Treatment of Cooley's anemia. Blood 1990; 76(3):435-444.
(7) Porter JB: Practical management of iron overload. Br J Haematol 2001; 115(2):239-252.
