Absolute Rates of Globin Chain Synthesis in Thalassemia

The absolute rate of α chain synthesis per erythroid cell in the peripheral blood of patients with β thalassemia has been shown to be normal while that of β chains is markedly decreased or absent. The results indicate that α chains do not require the presence of β chains for their normal synthesis and release. In addition, γ chain synthesis does not compensate for the decreased β chain synthesis. A marked heterogeneity in the amount of β globin chains produced by different patients with β thalassemia is also prominent.