Thalassemia Intermedia Due to Interaction of Lepore Trait with Thalassemia Trait

Abstract

The term thalassemia does not describe a single entity but rather a broad group of hereditary microcytic anemias with a characteristic abnormal erythrocyte morphology. The majority of these cases may be classified clinically into two groups: (1) thalassemia minor, a mild hypochromic microcytic anemia which is usually asymptomatic and is believed to be due to the heterozygous occurrence of a thalassemia gene; (2) thalassemia major, a severe progressive hemolytic anemia with marked abnormalities of the red cells, which is generally believed to be due to the homozygous occurrence of a thalassemia gene. However, there are cases which are not readily assigned to either of these categories. In addition to marked erythrocyte morphologic changes, these are characterized by a hemolytic anemia which is intermediate in severity and by splenomegaly, in most cases. Singer et al.¹ called this group thalassemia intermedia. Its etiology is varied. The development of hemoglobin electrophoretic techniques