Estrogen and Progesterone Receptors in Hereditary Hemorrhagic Telangiectasia

Abstract

Hereditary hemorrhagic telangiectasia (HHT) often requires transfusion and has major ill-effects. The recent literature reports successful high-dose estrogen treatment of epistaxis caused by HHT. To investigate this, biopsy specimens taken from areas clinically involved with telangiectasia in four patients were evaluated for estrogen- and progesterone-binding receptors. Specimens from two women (ages 34 and 38) were positive for both estrogen and progesterone receptors in ranges observed in breast carcinoma specimens. Specimens from two men (ages 34 and 78) were positive only for progesterone receptors at lower but clearly detectable levels of activity. Nasal mucosa specimens from control patients −2 male and 4 female – yielded no detectable levels of estrogen or progesterone receptors. Because of the side effects of high-dose estrogen (especially in males), we have initiated systemic progesterone therapy with both megestrol acetate and medroxyprogesterone acetate. Marked diminution in epistaxis incidence and severity was observed in three patients after initial systemic progesterone treatment. All treated patients have been maintained with good epistaxis control for over 1 year.