Thalassemia Syndromes

THE thalassemia syndromes, as the phrase implies, are a group of entities. It is now recognized that the clinical picture that one associates with thalassemia can arise from a variety of inherited disorders in hemoglobin formation. The unifying aspect of this problem from the laboratory point of view is a partial or complete depression in the formation of one of the hemoglobin polypeptide chains.I shall first consider in general terms the major types of thalassemia and then discuss recent studies that are leading to an understanding of the nature of the genetically determined defect in the β type of . . .