The parents of the propositus were characterized as heterozygotes for “normal HbA2, type 2 thalassemia”, which has a distinct hematological phenotype, with reduced red blood cells indices, decreased osmotic fragility and an unbalanced α/β-globin synthesis ratio comparable to that of heterozygotes with typical β0-thalassemia variants distinguished by increased HbA2 levels [3,4]. Here, HBA2 is linked to thalassemia.