Soluble and stroma-bound globin chains in mild and severe beta thalassemia

In mild beta thalassemia hemoglobin levels are much higher than in severe beta thalassemia, yet black patients with the mild syndrome are as deficient in beta chain synthesis as severe thalassemia patients. The following studies were undertaken to determine if differences in the precipitation of excess alpha chain distinguishes these two groups of beta thalassemia homozygotes. The percentages of radioactive globin chain bound to erythroid cell stroma were determined in mild and severe thalassemia patients. Only the cells of splenectomized severe patients contained large amounts of stroma-bound globin radioactivity, the percentages in five such patients ranging from 6.7 to 13.9 (mean = 9.9). In four splenectomized patients with mild thalassemia the percentages ranged from 1.0 to 3.5 (mean = 1.9; p less than 0.005). This is the first observed difference in globin chain metabolism between these two groups of patients.