pulmonary veno-occlusive disease

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

• bleomycin (6199283)

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. Lantuéjoul 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