Soluble and strana-bound globin chains in mild and severe β thalassemia

Abstract

In mild β thalassania henoglobin levels are much higher than in severe β thalassemia, yet black patients with the mild syndrome are as deficient in β chain synthesis as severe thalassemia patients. The following studies were undertaken to determine if differences in the precipitation of excess α chain distinguishes these two groups of β thalassemia homozygotes.

The percentages of radioactive globin chain hound to erythroid cell stroma were determined in mild and severe thalassemia patients. only the cells of splenectomized severe patients coontained large amounts of stroma-bound globin radioactivity, the percentages in five such patients ranging from 6.7 to 13.9 (x = 9.9). In four splenectomized patients with mild thalassemia the percentages ranged. fram 1.0 to 3.5 (x = 1.9; p<0.005). This is the first observed difference in globin chain metabolisn between these two groups of patients.