H-Y antigen in a 46,XX female with dysgenetic ovaries

Hypogonadism secondary to ovarian dysgenesis or resistant ovary syndrome was diagnosed in a 19-yr-old obese woman with primary amenorrhea, a 46,XX karyotype, and an H-Y+ cellular phenotype. Small ovoid gonads (1.5 X 0.6 cm) were found found bilaterally; these were encased in a dense venous network. The stroma was ovarian, and primordial follicles and some primary follicles were present, but there were no follicles at or beyond the antrum stage. There was no evidence of testicular tissue and no evidence of malignancy. Analysis of serological data indicated the possibility of residual H-Y antigen in the blood cells of the mother.