The association of hemoglobin Knossos and hemoglobin Lepore in an Algerian patient.
We report on a 54 years-old male patient from North-Eastern Algeria who combines two hemoglobin variants that are associated with thalassemia-like disorders: Hb Lepore and Knossos (beta 27 Ala----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(0)-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 hemoglobin, displayed a decreased affinity for oxygen (P50 = 35 mm Hg), a fact presumably accounting for the relatively good tolerance of the condition.
D000462:Algeria / Q000208:ethnology; D005602:France; D006455:Hemoglobins, Abnormal / Q000032:analysis* / Q000235:genetics; D006801:Humans; D007525:Isoelectric Focusing; D008297:Male; D008875:Middle Aged; D013156:Splenectomy; D013789:Thalassemia / Q000097:blood* / Q000235:genetics / Q000601:surgery
D006455:Hemoglobins, Abnormal; C036232:hemoglobin Knossos; C037659:hemoglobin Lepore