Hematologic changes in α-thalassemia

Abstract

Alpha-thalassemia is very common in Thailand. Interaction of the different types of α-thalassemia can lead to many α-thalassemia syndromes. In this study the authors compare the hematologic data of subjects with various α-thalassemia phenotypes. Designation of the genotypes was based on family study and DNA mapping. The results show that there are equivocal hematologic findings among those who have similar molecular defects i.e., α-thalassemia-2 and hemoglobin (Hb) Constant Spring heterozygotes: α-thalassemia 1, homozygous α-thalassemia 2, and α-thalassemia 2/Hb Constant Spring. The severity of these α-thalassemia syndromes correlates with the α-globin gene expression calculated from the finding of Liebhaber.