First Report of Known Rare RhNull Phenotype Individuals in Iran

Rhnull phenotype is a rare blood group with a frequency of approximately 1 in 6 million individuals, transmitted via an autosomal recessive mode. It is characterized by the weak (Rh mod) or lack (Rh null) of expression of all Rh antigens on the red cells. The clinical significance of its assessment is that such patients with Rhnull syndrome are associated with chronic hemolytic anemia of varying degrees. Another clinical importance is that such subjects readily form alloantibodies when exposed to Rh antigens.
We report herein a rare Rhnull phenotype in a sibling which was detected as a part of the difficult sample work-up for red cell antibody screening and identification.