Rh Deficiency Syndrome
The Rh deficiency syndrome, also known as Rh-null syndrome, is a blood disorder where people have red blood cells (RBCs) lacking all Rh antigens. The Rh antigens maintain the integrity of the RBC membrane and therefore, RBCs which lack Rh antigens have an abnormal shape. There are two types of Rh deficiency syndrome:
- The regulator type is associated with many different changes (mutations) in the RHAG gene .
- The amorph type is caused by inactive copies of a gene (silent alleles) at the RH locus.