RESUMEN
<p><b>OBJECTIVE</b>To investigate the clinicopathological features of pulmonary lymphangioleiomyomatosis (PLAM).</p><p><b>METHODS</b>By means of HE and immunohistochemistry (SP method) studies, the clinical and pathological features of 5 PLAM cases were analyzed and the related literature reviewed.</p><p><b>RESULTS</b>PLAM was a rare lung disease of unknown etiology and was restricted to females who were generally pre-menopausal. Pathological features showed abnormal smooth muscle cells (LAM cells) line the airways, lymphatics and blood vesssels leading to airflow obstruction and replacement of the lung parenchyma by cysts. LAM cells were positive for HMB45. Clinically the disease was categorized by dyspnoea, haemoptysis, recurrent pneumothoraces and chylous effusions.</p><p><b>CONCLUSIONS</b>PLAM should be considered when recurrent pneumothorax, haemoptysis and dyspnoea occur in females. Pathologic examination of lung tissue biopsy is required for confirmation of PLAM diagnosis.</p>