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April 1959

Thalassemia Intermedia Due to Interaction of Lepore Trait with Thalassemia Trait: Report of Three Cases

Author Affiliations

From the Department of Pediatrics, Harvard Medical School, and the Children's Medical Center.

AMA Am J Dis Child. 1959;97(4):464-472. doi:10.1001/archpedi.1959.02070010466011

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

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