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pulmonary veno-occlusive disease

pulmonary venoocclusive disease

Friday 12 March 2004

Pulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangiomatosis (PCH) are rare causes of pulmonary hypertension, regarded by some as distinct entities. However, their presentations are similar and both are associated with poor prognoses.

Synopsis (16819327)

- veinous intimal fibrosis
- arterial medial hypertrophy and/or intimal fibrosis (88%)
- hemosiderosis (79%)
- venulitis (12%)
- pulmonary infarction (9%)
- interstitial fibrosis (sometimes as localized scars) (48%)
- mild lymphocytic infiltrate (67%).

Etiology

- familial pulmonary veno-occlusive disease (7129408)
- post-chemotherapy

Associations

- pulmonary arterial occlusive disease (arterial intimal fibrosis) (4043952)

Ultrastructure (6341208)

- occluded pulmonary veins

  • intact endothelial cells
  • haphazard proliferation of collagen fibrils and smooth muscle cells

- alveolar capillaries

  • thickening of the endothelial cell basement membrane
  • increase in the number of cytoplasmic processes of pericytes
  • electron-dense deposits located within the thickened basement membrane (considered to represent disintegrating extravasated erythrocytes rather than immune complexes)

See also

- pulmonary capillary hemangiomatosis (PCH)

References

- Pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis: a clinicopathologic study of 35 cases. LantuĂ©joul S, Sheppard MN, Corrin B, Burke MM, Nicholson AG. Am J Surg Pathol. 2006 Jul;30(7):850-7. PMID: 16819327

- Carrington CB, Liebow AA. Pulmonary veno-occlusive disease. Hum Pathol. 1970 Jun;1(2):322-4. PMID: 5521729