Other non-neoplastic 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.
● 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
● 6 year old girl with progressive dyspnea (Arch Pathol Lab Med 2003;127:e393)
● 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
Fig 3: septal veins; Fig 4: elastin stain
● UIP: no increased elastic fibers
End of Lung-nontumor > Other non-neoplastic disease > Veno-occlusive disease
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