Diffuse pulmonary lymphangiomatosis: Pulmonary lymphatic disorder in an adult

Abstract

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.

A Rare Case of Diffuse Pulmonary Lymphangiomatosis in a Middle-Aged Woman

“…Surgical resections can be indicated for localized mediastinal or lung lesions. Other treatments include low-fat medium-chain triglyceride diets, interferon-alpha, radiation, corticosteroids, chemotherapy, somatostatin, and propranolol ( 2 ).…”

“…Patients with DPL present with various clinical manifestations and usually have a progressive clinical course (2). …”

“…An increase in the size and the number of anastomosing lymphatic channels in interlobular septa or subpleural areas is seen histopathologically ( 2 ). Patients with DPL present with various clinical manifestations and usually have a progressive clinical course ( 2 ).…”