Multiple Inherited Erythrocyte Abnormalities in an American Negro Family: Hereditary Spherocytosis, Sickling and Thalassemia

An American Negro family has been described in which there are found the genes responsible for three inherited abnormalities of the erythrocyte, namely, the genes resulting in spherocytosis, a thalassemia-like trait and the sickling phenomenon. The study of this family provides evidence for the independent segregation of the genes responsible for this type of thalassemia and the sickling phenomenon, and for spherocytosis and the sickling phenomenon. It is noteworthy that the thalassemia gene present in this family is not associated with an increased proportion of hemoglobin A₂, and fails to exhibit "factor interaction" with the sickle cell gene, and the question is raised whether these attributes characterize a type of thalassemia gene non-allelic to the sickle cell gene.