Abstract

Lymphangioleiomyomatosis (LAM) is a disease that predominantly affects young females and generally progresses to respiratory failure and death. A 33-year-old postpartum woman was admitted with progressive dyspnea and high CA-125 levels. She had bilateral chylopneumothorax, chylous ascites and was diagnosed as LAM by open lung biopsy. Her management was very complex as her chylous ascites and pleural effusions were very resistant to therapy. Betadine pleurodesis was very successful in the management of chylothorax. There were no LAM cases presenting with high CA-125 levels. We believe that CA-125 levels could be used as a useful parameter in patient follow-up. (Tur Toraks Der 2011; 12: 127-30)