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

Abstract

We report on a 54 years-old male patient fran North-Eastern Algeria who carbines tm hemoglobin variants that are associated with thalassemia-like disorders : Hb Lepore and Knossos (β27 Ala ô Ser) (1, 2). A β-thalassemia intermedia picture qradually developed and finally required splenectomy at the age of 53. Total absence of Hb A₂ indicated that the β knossos gene is most probably flanked with a δ⁰-⁻thalassemia gene. No DNA deletion additional to the Lepore deletion was found. Hb F was elevated (12.3 %) with 24 % ^Gγ Hb F. In whole cells, Hb Knossos, representing 70% of total hemoglobin, displayed a decreased affinity for oxygen (P₅₀ = 35 mm Hg), a fact presumably accounting for the relatively good tolerance of the condition.