Abstract
Diffuse pulmonary lymphangiomatosis (DPL) is rare in adults. It is characterized by
abnormal proliferation, dilatation, and thickening of the lymphatic channels in the
lungs, pleura, and mediastinal soft tissue. Here, we report a case of DPL in a young
adult man with recurrent productive cough. Chest computed tomography (CT) showed bilateral
interlobular septal and peribronchovascular thickening and mediastinal soft tissue
infiltration. Lung biopsy through video-assisted thoracic surgery demonstrated proliferation
and dilatation of irregular lymphatic spaces, lined by flattened endothelial cells
that were positive for CD31, D2-40, and factor VIII-related antigen on immunohistochemical
staining. After treatment with propranolol for six months, the chest CT showed improved
interlobular septal and peribronchovascular thickening and a unilateral pleural effusion,
which turned bloody. Radiologic features can suggest the diagnosis of DPL. Surgical
biopsy with adequate section size is critical in the diagnosis. Propranolol might
be an effective and safe therapeutic option for patients with DPL.
Key Indexing Terms
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Article info
Publication history
Published online: April 08, 2022
Accepted:
March 30,
2022
Received:
May 4,
2021
Identification
Copyright
© 2022 Southern Society for Clinical Investigation. Published by Elsevier Inc. All rights reserved.
