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Diffuse Lung Disease: Diffuse Lung Disease |

An Adult Case of Diffuse Pulmonary Lymphangiomatosis FREE TO VIEW

Yuqian Li, PhD; Zhancheng Gao, PhD
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Peking University People's Hospital, Beijing, China


Copyright 2016, American College of Chest Physicians. All Rights Reserved.


Chest. 2016;149(4_S):A203. doi:10.1016/j.chest.2016.02.210
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SESSION TITLE: Diffuse Lung Disease

SESSION TYPE: Case Report Slide

PRESENTED ON: Saturday, April 16, 2016 at 05:15 PM - 06:15 PM

INTRODUCTION: Diffuse pulmonary lymphangiomatosis (DPL) is a rare lymphatic disorder, occurring mostly in children and young adults without gender predilection. It is a progressive and fatal disease. It is histologically observed that there is an increase in the diameter and number of involved lymphatics.

CASE PRESENTATION: A 53 year old woman was admitted to hospital for further evaluation of mediastinal widening and ground-glass opacities on her CT. These abnormalities had first been noticed 1 month previously, when she got a cold and felt a bit out of breath. In addition to mild short of breath, she had no other complains. Her vital signs are stable, she had no positive signs, except for some crackles in the bottom of the lung. CT showed diffuse soft tissue infiltration in the mediastinum without displacement of the vessels. Patchy bilateral ground-glass opacities and bilateral smooth thickening of the interlobular septa and bronchovascular bundles were observed in the lung parenchyma. Bronchoscopy showed mucosal inflammation with congestive and edema of the mucosa, The tracheal carina was sharp during inspiratory phase, while was blunt during expiratory phase. The airway wall was also the same. Pulmonary and mediastinal biopsies were taken by means of video-assisted thoracoscopy and mediastinoscopy. Pathological examination showed an increase in the number of lymphatic channels in the lung parenchyma, lined by flattened endothelial cells strongly positive for CD31 on immunohistochemical study. Fibrous tissue components were observed in the Mediastinal lymph nodes. The pathology leading to the definitive diagnosis of DPL.

DISCUSSION: The diagnosis of DPL can be suggested clinically and with proper imaging. Pathological examination can distinguish the disease from other disease; the proliferation of lymphatic vessels in the pleura and interlobular septa and peribronchovascular connective tissue is the key finding. Individuals suspected of this should do biopsy as soon as possible.

CONCLUSIONS: DPL is a rare disease to be considered in the differential diagnosis of pulmonary lymphatic disorders, and is characterized diffuse interlobular septal thickening, pleural effusion, and mediastinal soft tissue infiltration.

Reference #1: Tazelaar HD, Kerr D, Yousem SA, Saldana MJ, Langston C, Colby TV. Diffuse pulmonary lymphangiomatosis. Human Pathol 1993; 24:1313-1322.

Reference #2: Ramani P, Shah A. Lymphangiomatosis: histologic and immunohistochemical analysis of 4 cases. Am J Surg Pathol 1993;17:329-35.

Reference #3: Swensen SJ, Hartman TE, Mayo JR, et aI. Diffuse pulmonary lymphangiomatosis: CT findings. Journal of Computer Assisted Tomography 1995;19(3):348-52.

DISCLOSURE: The following authors have nothing to disclose: Yuqian Li, Zhancheng Gao

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