Hematological and biosynthetic features of β⁰-thalassemia with an associated α-thalassemia heterozygosity in Sardinia

Abstract

Total hemoglobin, PCV, hemoglobin A2 and hemoglobin F were determined in controls, β0-thal heterozygotes and α-thal+ β thal heterozygotes. Also the distribution of mean corpuscular volume and the mean corpuscular hemoglobin values in Sardinian β0-thal heterozygotes with and without an associated α-thal and the ratio of α/β chain synthesis in β0-thal heterozygotes with or without an associated α-thal were studied