Blood, 1960, Vol. 15, No. 5, pp. 610-613.
© 1960 American Society of Hematology, Inc.
The Hemoglobin E Syndromes. II.
Sickle-Cell—Hemoglobin E Disease
MUZAFFER AKSOY 1
1 Beyoglu Ilk Yardim Hospital, Istanbul, Turkey.
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.
