Pulmonary Lymphangioleiomyomatosis:

47-year-old woman is presented with pulmonary lymphangioleiomyomatosis (PLAM)involving the bilateral lung and slight pulmonary function abnormality. Computed tomography scan showed bilateral microcyst formation in the lung. Histologicaly,proliferating spindle shaped cels with centrilobular emphysema were main findings.Immunohistochemicaly,these proliferating spindle shaped cels were positive forα-smooth muscle actin,desmin,vimentin,HMB45,estrogen receptor and progesterone receptor,but negative for S-100,cytokeratin.Single strand conformation polymorphism(SSCP)and DNA analysis for tuberous sclero-sis 1 and 2 showed no significant abnormality.――――pulmonary lymphan-gioleiomyomatosis;HMB45?2003 Tohoku University Medical Press Pulmonary lymphangioleiomyomatosis (PLAM)is a rare disease that occurs almost exclusively in women of reproductive age (Bonetti et al.1993).It is manifested by HMB45 positive smooth muscle proliferatio