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Case Reports
. 2013 Jan-Feb;20(1):52-4.
doi: 10.1155/2013/971350.

Diffuse pulmonary lymphangiomatosis

Kunal C Kadakia  1 , Sandeep M Patel, Eunhee S Yi, Andrew H Limper
Affiliations
Case Reports

Diffuse pulmonary lymphangiomatosis

Kunal C Kadakia et al. Can Respir J. 2013 Jan-Feb.

Abstract
in English, French

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.

La lymphangiomatose pulmonaire diffuse (LPD) est une maladie rare caractérisée par l’infiltration de lymphangiomes à paroi mince dans les poumons, la plèvre et le médiastin. La LPD peut entraîner un effet de masse causé par la maladie infiltrante, la physiologie pulmonaire restrictive et obstructive, les effusions chyleuses et l’insuffisance respiratoire. Le présent article aborde les caractéristiques cliniques, radiographiques et pathologiques, de même que les possibilités thérapeutiques, de la LPD.

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Figures

Figure 1)
Figure 1)
Anteroposterior chest x-ray showing upper-lobe predominant interstitial infiltrates and bilateral pleural effusions
Figure 2)
Figure 2)
Computed tomography scan of the chest revealing peribronchovascular and interlobular thickening, interstitial infiltrates and pleural thickening with effusions
Figure 3)
Figure 3)
Computed tomography of the chest (mediastinal window) showing diffuse infiltration of the mediastinum by cystic fluid densities (arrows)
Figure 4)
Figure 4)
Low-power view demonstrates a markedly thickened visceral pleura and interlobular septa due to abnormal lymphatic vascular proliferation (hematoxylin and eosin stain, original magnification ×20), as supported by D2-40 immunohistochemical staining (inset, original magnification ×200)
See this image and copyright information in PMC

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