Lung-nontumor - Veno-occlusive disease

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Lung-nontumor

Other non-neoplastic disease

Veno-occlusive disease

Reviewers: Elliot Weisenberg, M.D. (see Reviewers page)
Revised: 19 December 2011, last major update December 2011
Copyright: (c) 2003-2011, PathologyOutlines.com, Inc.

Clincal features
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● 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
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● 6 year old girl with progressive dyspnea (Arch Pathol Lab Med 2003;127:e393)

Micro description
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● 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

Micro images
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Fig 3: septal veins; Fig 4: elastin stain

Various images

Differential diagnosis
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● UIP: no increased elastic fibers

End of Lung-nontumor > Other non-neoplastic disease > Veno-occlusive disease

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