A case of fatal hemolytic disease of the newborn associated with-D-/-D-phenotype.

-D- is a rare haplotype that determines D without C, c, E or e, and exalted D activity. The extremely rare homozygote propositi (-D-/-D-) are usually ascertained through their immune antibodies, anti-Rh17 (Hro), which react with red cells of all common Rh phenotypes. The authors experienced the first case in Korea of a woman with -D- phenotype. She had a history of spontaneous abortion, therapeutic termination and red cell transfusion, and at her third pregnancy she delivered a baby with severe hemolytic disease of the newborn. In spite of intensive medical intervention, the baby died of hydrops fetalis. An immune antibody to high incidence Rh antigen, namely anti-Rh17(Hro), was demonstrated in the woman's serum. A family study revealed that the -D- gene complex was present in all its members and one of the woman's sisters was also -D- homozygote. In the serum of this sister, anti-Rh17(Hro) was also present.