| Abstract: | In a new subclass of G gamma HPFH which has been detected in a black family, beta A chains are produced in cis to the HPFH determinant (the G gamma-beta+ HPFH). No other instance of beta chain production in cis to HPFH has been reported. All individuals in this family are well even if Hb S is produced in trans to HPFH. Genetically, this new subclass requires a slightly smaller deletion in the gamma, delta, and beta complex of genes than do other forms of HPFH. It is speculated that a subclass (the G gamma-(G gamma A gamma)-beta+ HPFH) in which beta S chains are produced in cis to HPFH in conjunction with true beta S genes in trans may be responsible for "mild" cases of sickle cell anemia. |
