OVARIAN RESISTANCE TO LUTEINIZING-HORMONE - A NOVEL CAUSE OF AMENORRHEA AND INFERTILITY

Objective: To report the clinical, hormonal, and histopathological fea tures of a woman with ovarian resistance to LH. Design: Clinical study . Setting: University hospital. Patient(s): A woman with amenorrhea, s ister of a patient with male pseudohermaphroditism due to Leydig cell hypoplasia. Intervention(s): Blood drawing before and after GnRH stimu lation and also after dexamethasone and hCG administration, pelvic ult rasound, and ovarian biopsy.Main Outcome Measure(s): Karyotype, gonado tropin and steroid measurements, follicular diameter, ovarian histolog y, and sequencing of the LH receptor gene. Result(s): Patient had norm al female external genitalia, normal breast development at puberty, ra re episodes of vaginal bleeding, and infertility. The karyotype was 46 ,XX. She had elevated serum LH levels, whereas E(2) and P concentratio ns were in the range seen in the early follicular phase. Pelvic ultras ound revealed a slightly hypoplastic uterus and enlarged polycystic ov aries. A normal follicular reserve for age, antral follicles, and abse nce of corpora lutea or albicans were observed on ovarian biopsy. Exon 11 of the LH receptor gene had a normal sequence. Conclusion(s): In o ur patient with ovarian resistance to LH, FSH stimulated follicular de velopment until the preovulatory stage, but E(2) levels remained in th e early follicular phase range, still sufficient for normal pubertal f eminization. Apparently, LH is necessary for ovulation and corpus lute um formation.