The First Observation of Sickle-Cell Hæmoglobin E Disease

Abstract

IT is likely that adult hæmoglobin (hæmoglobin A) and its variants are all allelomorphs, but strongly supporting evidence has so far been produced only that hæmoglobins A, S and C are allelomorphs¹. The Eti-Turks are a small Arabic-speaking group in southern Turkey with a high incidence of the sickling gene². When, in 1955, an Eti-Turk was discovered with both hæmoglobins A and E ³, we hoped that a family might be found in this community in which there was a segregation for hæmoglobins A, S and E and where we might observe a new sickling condition, sickle-cell – hæmoglobin E disease. Although we examined many Eti-Turks, no further cases of hæmoglobin E were discovered. We therefore thought that hæmoglobin E might perhaps be found in the Turks themselves, and that the observation on a single Eti-Turk might have been the outcome of a miscegenation. We investigated more than 400 Turks; but although we found one family with hæmoglobin D ⁴, no further instances of hæmoglobin E came to light.