The Association of Hemoglobin Knossos and Hemoglobin Lepore in an Algerian Patient

Abstract

A 54 yr-old male patient from North-Eastern Algeria was reported who combines 2 Hb variants that are associated with thalassemia-like disorders: Hb Lepore and Knossos (.beta.27 Ala .fwdarw. Ser) (1, 2). A .beta.-thalassemia intermedia picture gradually developed and finally required splenectomy at the age of 53. Total absence of Hb A2 indicated that the .beta. Knossos gene is most probably flanked with a .delta..degree.-thalassemia gene. No DNA deletion additional to the Lepore deletion was found. Hb F was elevated (12.3%) with 24% G.gamma. Hb F. In whole cells, Hb Knossos, representing 70% of total Hb, displayed a decreased affinity for O2 (P50 [O2 tension at 50% saturation Hb] = 35 mm Hg), a fact presumably accounting for the relatively good tolerance of the condition.