Synthesis of Globin Chains in Sickle β-Thalassemia

• Text
• PDF

Abstract

In five patients with sickle β-thalassemia there was balanced α- and β-globin synthesis in the bone marrow and decreased total β-chain synthesis relative to that of α-chain in the peripheral blood. These findings are similar to those in patients with simple β-thalassemia trait. Despite a range of hemoglobin concentrations from 6.8 to 12.5 g/100 ml in the patients with sickle thalassemia, there was no evidence of a significant excess of α-chains in the red cells of the bone marrow which could contribute to the hemolysis and anemia.

Authors

Frances M. Gill, Elias Schwartz