ALPHA-ALPHA-ALPHA-ANTI-4.-2 HAPLOTYPE AND HETEROZYGOUS BETA-O THALASSEMIA IN A SICILIAN ITALY FAMILY

The presence of the .alpha..alpha..alpha.anti-4.2 haplotype and heterozygous .beta..degree. thalassemia in a Sicilian family is described. These findings confirm the presence in Italy of a leftward deletion (-.alpha.4.2) and indicate that this may not be rare. Furthermore, although the .beta. thalassemia determinant in this family has a severe expression, the interaction with the triplicated .alpha. gene does not necessarily express itself as thalassemia intermedia.