Poster Session : PS 0643 ; Respiratory Medicine ; Successful Treatment of Diffuse Pulmonary Lymphangiomatosis with Propranolol = Poster Session : PS 0643 ; Respiratory Medicine ; Successful Treatment of Diffuse Pulmonary Lymphangiomatosis with Propranolol

다국어 초록 (Multilingual Abstract)

Introduction: Diffuse pulmonary lymphangiomatosis (DPL) is a rare congenital disease, which shows the overgrowth of lymphatic vessels in the lungs, pleura, and mediastinum. Although DPL has often poor prognosis, treatment options including surgery, interferon, radiotherapy, and glucocorticoids are limited. We report on a patient with DPL, who was treated with propranolol (non-selective beta blocker) successfully. Case: A previously healthy 53-year-old man visited our hospital with cough and shortness of breath. He underwent chest computed tomography (CT) examination and it showed a large amount of pleural fi uid on left side with crazy paving pattern on both lung fi elds. The pleural fi uid showed chylous effusion with the triglyceride level of 1288 mg/dL. We performed a video associated thoracospcopic surgery (VATs) lung biospy for diagnosis and a pleurodesis with talc. Microscopically, the obtained tissue showed the dilated lymphatic channels growing in connective tissue and these lymphatic channels revealed positive D2-40 staining immunohistochemically. Under the diagnosis of DPL, the patient was treated with a low-fat medium-chain triglyceridediet and discharged with improved symptoms. One month later after his discharge, he complained of mild cough and dyspnea again. Follow-up chest CT revealed more extended crazy paving appearance on both lungs with recurred pleural effusion. Oral propranolol, which had been proposed as an alternative treatment for lymphatic malformation, was attempted to administrate at 0.5 mg/kg/day initially, then it was increased to 1.5 mg/kg/day. 5 months after treatment with propranolol, the patient presented relieved respiratory symptoms with slightly improved ground glass appearance and interlobular septal thickening on follow-up chest CT.Discussion: There are no established curative treatments available for DPL. Our patient had clinical improvement for respiratory symptoms and radiologic aspect after administering propranolol. As a conclusion, we expect that our report contributes to a strategy of DPL.