• Tuberculosis and Respiratory Diseases
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• v.70 no.5
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• pp.428-432
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• 2011

Pulmonary lymphangiomatosis is a rare disorder involving the entire intrathoracic lymphatic system from the mediastinum to the pleura. Pulmonary lymphangiomatosis mostly occurs in children and young adults without gender predilection. Although it is pathologically benign, it shows a progressive and fatal course with variable initial presentation. We now report a case of pulmonary lymphangiomatosis in a 35-year-old man. He presented with hemoptysis 6 months previously. Chest x-ray and a chest computed tomography scan showed diffuse interstitial thickening with left pleural effusion. Chylothorax was confirmed by thoracentesis. Lymphangiography showed dilated and tortuous lymphatic channels. Surgical lung biopsy revealed proliferation of complex anastomosing lymphatic channels. He was diagnosed with pulmonary lymophangiomatosis. Closed thoracostomy and chemical pleurodesis were done and the dyspnea was reduced.

• Tuberculosis and Respiratory Diseases
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• v.47 no.4
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• pp.533-537
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• 1999

Pulmonary lymphangiomatosis is a very rare pulmonary lesion with an aggressive potential that occurs mainly in newborns, infants and young children of both sexes. It is characterized by pulmonary abnormalities of lymphatic system, showing an increased number of complex anastomosing lymphatic channels in the pleura, in the subpleural interlobular septa, and along the bronchovascular lymphatic route and uniformly fatal. We report a case of lymphangiomatosis behaving like lymphangioleiomyomatosis in a 26-year-old woman.