[Pulmonary lymphangiomyomatosis: report of a case]

A case of pulmonary lymphangiomyomatosis in a 28-year-old woman with dyspnea, hemoptysis, chylothorax and irregular menstruation as presenting symptoms was reported. Chest CT scan showed the presence of pleural effusion on right side and a honeycomb pattern throughout the lung parenchyma bilaterally. The diagnosis was confirmed histologically by transbronchial lung biopsy. Medroxyprogesterone was administered; improvement in arterial blood gas analysis and reduction of the amount of pleural effusion were noted after one month. The etiology, clinical manifestations, pathological characteristics and treatment of this disease were discussed.