Diffuse Pulmonary Lymphangiomatosis

Diffuse Pulmonary Lymphangiomatosis

Monica P. Revelo, MD, PhD

Brandon T. Larsen, MD, PhD

Numerous irregular lymphatic channels image expand the pleura but preserve the underlying alveolated lung parenchyma image. In some cases, hemorrhage image into the lymphatic spaces may be present.

Irregular, thin-walled, anastomosing lymphatic channels dissect between pleural collagen bundles. On higher power, the bland endothelial cell lining image of the lymphatic spaces is apparent.



Developmental Anomaly

  • Congenital disease due to abnormal lymphatic system development



  • Age

    • Mean at presentation: 8.5 years

    • May manifest at birth and rarely in adults

  • Gender

    • No predilection


  • Localized to lung or chest

  • Widespread distribution involving spleen, bones (e.g., ribs, vertebrae, humerus, femur), liver, and skin


  • Dyspnea

  • Cough

  • Hemoptysis

  • Chest pain

  • Milky sputum

  • Pleural effusions

  • Mediastinal enlargement

Laboratory Tests

  • Pulmonary functional tests demonstrate

    • Mixed pattern obstructive/restrictive

    • Restrictive pattern only

Natural History

  • Progressive condition leading to end-stage respiratory failure


  • Poor; causes of death include respiratory failure, infections, and chylus effusions


Radiographic Findings

  • Chest x-ray shows mediastinal enlargement

  • Peribronchial expansion

  • Increased interstitial markings

  • Chylous pleural/pericardial effusions

  • Multifocal cystic bone lesions with porous soap or honeycomb texture

CT Findings

  • Thickening of interlobular septa and bronchovascular bundles

  • Ground-glass opacities

  • Pleural thickening and effusions

  • Diffuse fluid infiltration of mediastinal soft tissue

  • Increased mediastinal/hilar nodes

Jul 8, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Diffuse Pulmonary Lymphangiomatosis

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