Erythropoiesis and the Effect of Transfusion in Homozygous β-Thalassemia

THE erythropoietic abnormality in patients with homozygous β-thalassemia results from a genetically determined defect in globin (β-chain synthesis. Erythroid activity is vastly increased, and estimates of the degree of hyperplasia vary from five to 30-fold.1 , 2 Much of this activity is known to be ineffective, but quantitative assessment of total, effective and ineffective erythropoiesis has not been possible. We have used a recently developed ferrokinetic technic3 , 4 to measure the amount of erythropoiesis and its effectiveness in patients suffering from homozygous β-thalassemia, and this procedure has allowed a preliminary examination of the effect of blood transfusion on erythroid activity. In patients having . . .