The Hemoglobin E Syndromes. II. Sickle-Cell—Hemoglobin E Disease

1. A clinical and hematologic description is presented of a patient with sickle-cell-hemoglobin E disease in a new family. In contrast to the mild hematologic and clinical changes observed in the patients described by the author previously, the clinical and hematologic manifestations in the new patient were moderate in degree. The anemia was normocytic in type. Variation in the clinical and hematologic picture of sickle cell-hemoglobin E disease has been noted.

2. Genetic studies of two families with sickle-cell-hemoglobin E disease have been presented. According to the genetic data obtained by evaluation of the genealogies of these two families, it is strongly suggested that the genes responsible for hemoglobins S, E and A are allelomorphs or linked.