Association of Hb e with β° Thalassemia

Abstract
Hb Hope .beta. 136 (H 14) Gly .fwdarw. Ala is a mildly unstable Hb variant initially described in 1965 by Minnich et al. in an American Negro family. Other cases were since reported among African and American Blacks. The O2 affinity, the cooperativity and the 2,3 diphosphoglycerate (2,3 DPG) interaction of this mutant were decreased but the Bohr effect was normal. Associations of this abnormal Hb with Hb S and with .beta.+ thalassemia were described. A case of Hb Hope associated with .beta.o thalassemia is reported. This is the 1st example in which the major Hb component within the RBC [red blood cell] was Hb Hope.