Diffuse pulmonary lymphangiomatosis: Pulmonary lymphatic disorder in an adult

Diffuse pulmonary lymphangiomatosis (DPL) is a rare disorder involving the intrathoracic lymphatic system from the mediastinum to the pleura. Although DPL is a lymphatic developmental abnormality and is pathologically benign, it has various clinical presentations and a progressive course. We recently studied a case of DPL in a 35-year-old man presenting with cough and hemoptysis with unilateral chylothorax. A chest computed tomography scan showed diffuse interstitial thickening along the peribronchovascular bundles and intralobular septae with pleural and pericardial effusion. Wedge resection of the left lower lung was performed to make a diagnosis. Microscopically, the lesion was characterized by variably sized and increased lymphatic channels of the interlobular septa and subpleural area. The cells lining the lymphatics were immunohistochemically positive for D2–40 and CD34. This patient is still alive without recurrent symptoms 13 months after beginning a low-fat medium-chain triglyceride diet and chemical pleurodesis.