Agonadism with positive H-Y antigen.

A 30-year-old phenotypic female was investigated because of absence of the vagina. Her serum gonadotropins were elevated while testosterone was in the normal range for females. Stimulation with human chorionic gonadotropin (hCG) gave no response of testosterone. No uterus, Fallopian tubes, Wolffian derivatives, or the gonads were found at laparotomy. Chromosome analysis showed a 46,XY karyotype. The expression of the H-Y antigen was positive. The results suggest that testicular degeneration in agonadism is not caused by a defective H-Y antigen.