Abstract
Diffuse pulmonary lymphangiomatosis (DPL) is a rare disease characterized by infiltration of the lung, pleura and mediastinum with thin-walled lymphangiomas. DPL can result in mass effect from infiltrative disease, restrictive and obstructive pulmonary physiology, chylous effusions and respiratory failure. The present article discusses clinical, radiographic and pathological features, and treatment options for DPL.
Highlights
Diffuse pulmonary lymphangiomatosis (DPL) is a rare disease characterized by infiltration of the lung, pleura and mediastinum with thin-walled lymphangiomas
Lymphangiomas are congenital, benign, cystic, focal areas of lymphatic proliferation that can occur in any part of the body containing lymphatics
Widespread proliferation of these anastomosing lymphatic spaces limited to the thorax leads to the rare syndrome of DPL [1]
Summary
Diffuse pulmonary lymphangiomatosis (DPL) is a rare disease characterized by infiltration of the lung, pleura and mediastinum with thin-walled lymphangiomas. DPL can result in mass effect from infiltrative disease, restrictive and obstructive pulmonary physiology, chylous effusions and respiratory failure. The present article discusses clinical, radiographic and pathological features, and treatment options for DPL. Radiographic and pathological features of diffuse pulmonary lymphangiomatosis (DPL). What are the common clinical features of DPL? 2. What treatments are available for the management of this disease?
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