Haemoglobin Hasharon in a north Italian community.

Hemoglobin Hasharon [<i>α</i>₂ 47 (CD5) Asp→ His β₂]Linked to <i>α</i>-Thalassemia in Northern Italian Carriers

“…The existence of an a-thalassemia-2 determinat in cis to the aHash locus would ac count for the surprisingly high frequency of Hb Hasharon trait observed in the northern moiety of the Polesine region, which was previously reported to be about 0.8%> [1]. Our more recent estimate deriving from a survey of 10,545 individuals in the province of Rovigo indicates an incidence of 0.23%, which appears to be more accurate.…”

“…8 out of these 43 families had already been identified in a previous report [1]. The abnormal Hb carriers were identified by routine cellulose acetate electrophoresis of hemolysates during //-thalassemia screening programs carried out on schoolchildren living in the prov ince of Rovigo (Polesine), followed by family studies.…”

“…A minor Hb component (presumably Hb Hash.,), migrating more slowly to the anode than Hb A,, was also detected. Globin chain electrophoresis indi cated the presence of slow-moving abnor mal a-chains, the subsequent structural characterization of which indicated that it was identical to aUasharon-chain [a 47 (CD5) Asp -* His], as previously described [1]. Structural analysis was always carried out at least on one carrier from each family.…”

“…Since its discovery in a Hebrew family of Ashkenazy origin [8], it was found in a number of carriers belonging to two main ethnical groups: (i) eastern European or Ashkenazy Hewbres, in whom the amount of Hb Hasharon ranged from 14 to 22°/o of the total Hb [reported by Lehmann and Vella,12]; (ii) north Italians, in whom Hb Hasharon accounted for 3 0 -4 0 % of the total Hb [1], a level significantly higher than that found in Ashkenazy carriers.…”