Lung - nontumor
Other nonneoplastic disease
Veno-occlusive disease

Topic Completed: 1 December 2011

Revised: 30 January 2019, last major update December 2011

Copyright: (c) 2003-2018,, Inc.

PubMed search: veno-occlusive disease [title] pulmonary

Elliot Weisenberg, M.D.
Page views in 2019: 550
Page views in 2020 to date: 210
Cite this page: Weisenberg E. Veno-occlusive disease. website. Accessed May 31st, 2020.
Clinical features
  • Rare, usually children and adolescents, especially females
  • Also after cancer chemotherapy
  • Causes pulmonary hypertension secondary to widespread thromboemboli of large and medium size branches of pulmonary vein; also diffuse occlusion of postcapillary venous beds by fibrous tissue
  • Usually recanalization and pseudoangiomatous changes, arterial thickening and hemosiderosis
  • Immune complexes may be involved in pathogenesis
  • Most patients die within 2 years of diagnosis; lung transplant may prolong lives
Case reports
Microscopic (histologic) description
  • Eccentric intimal thickening of venules in lobular septa with some septal veins completely occluded, increased elastic fibers in venous media (highlighted with elastin stain)
  • Medial hypertrophy of arterioles, but no plexiform lesions
  • Intraalveolar hemorrhage and hemosiderosis
  • Prominent alveolar septa due to congestion
Microscopic (histologic) images

Images hosted on other servers:

Fig 3: septal veins; Fig 4: elastin stain

Differential diagnosis
  • UIP: no increased elastic fibers
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