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Canadian Respiratory Journal
Volume 20 (2013), Issue 1, Pages 52-54
Clinico-Pathologic Conferences

Diffuse Pulmonary Lymphangiomatosis

Kunal C Kadakia,1 Sandeep M Patel,1 Eunhee S Yi,2 and Andrew H Limper3

1Division of Internal Medicine, Mayo Clinic, Rochester, Minnesota, USA
2Department of Anatomic Pathology, Mayo Clinic, Rochester, Minnesota, USA
3Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, Minnesota, USA

Copyright © 2013 Hindawi Publishing Corporation. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


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.