Pulmonary lymphangioleiomyomatosis in a postmenopausal woman: case report with review of literature.

Abstract

Lymphangioleiomyomatosis (LAM) is a rare disorder that occurs almost exclusively in young females and characterized by peribronchial, perivascular, and perilymphatic non-neoplastic proliferation of smooth-muscle cells. These changes may precipitate chylous effusions, pulmonary hemorrhage, bronchial cyst formation, progressive loss of lung function, and ultimately death. Most patients die within eight to ten years of the time of diagnosis. We report a rare case ofpost-menopausal onset pulmonary lymphangioleiomyomatosis in a 62-year old Caucasian female that was diagnosed at age 53 and responded well to intramuscular injections of Depo-Provera and bilateral oophorectomy. No symptom progression or pulmonary function test deterioration was noted over an eight year period of clinical follow-up. Our case is unique because of the postmenopausal onset and the unusual clinical course of the disease.