Is blood transfusion therapy the ideal treatment for -thalassemia intermedia?

The thalassemias represent the most common single gene disorder worldwide. The total annual incidence of symptomatic individuals with β-thalassemia is estimated at 1 in 100,000 throughout the world [1] of which nearly 10% have β-thalassemia intermedia (TI) [2]. Several approaches have been used for treating patients with TI including blood transfusions, splenectomy, iron chelation therapy, modulation of foetal haemoglobin and other agents focusing on specific aspects of clinical presentation. However the exact management of the disease is still controversial. A major issue which is not entirely explored is the effect of blood transfusion in the management of patients with β-thalassemia intermedia. There are certain advantages and disadvantages of initiating blood transfusions in TI patients that must be always taken into account in the management of these patients. Therefore, complications that interact with the clinical outcome of treatment and also patient’s quality of life can be avoided to some extend.References:Galanello R, Origa R. Beta-thalassemia. Orphanet J Rare Dis. 2010; 5: 11.Rachmilewitz EA, Giardina PJ. How I treat thalassemia. Blood. 2011; 118: 3479-88.