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Pathology of pulmonary veno-occlusive disease and pulmonary capillary haemangiomatosis 

Pathology of pulmonary veno-occlusive disease and pulmonary capillary haemangiomatosis
Chapter:
Pathology of pulmonary veno-occlusive disease and pulmonary capillary haemangiomatosis
Author(s):

Clive Handler

and Gerry Coghlan

DOI:
10.1093/med/9780199572564.003.006
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date: 14 October 2019

PVOD 30

PCH 30

PVOD is a rare pulmonary vascular disease causing PH. The incidence is 0.1–0.2 per million per year. The preseptal and septal venules are affected and become fibrotic and thickened due to cellular proliferation.

The characteristic findings are: occlusive fibrotic lesions, venous muscularization, patchy capillary proliferation, pulmonary oedema, lymphatic dilatation, lymph node enlargement, and inflammatory infiltrates. Occult pulmonary haemorrhage is common in PVOD probably due to post-capillary block....

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