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Pulmonary veno-occlusive disease

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Pulmonary veno-occlusive disease

Definition:

Pulmonary veno-occlusive disease is a rare form of primary pulmonary hypertension (high blood pressure in the blood vessels of the lungs) in which progressive obstruction of the pulmonary veins leads to elevated blood pressure in the arteries of the lungs.

Alternative Names:

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Pulmonary vaso-occlusive disease

Causes, incidence, and risk factors:

In most cases, the cause is unknown, but it may be related to a viral infection. It may occur as a complication of certain connective tissue diseases, such as lupus or CREST, or as a complication of certain blood cancers (leukemia or lymphoma) or chemotherapy.

The disorder is most common amongst children and young adults. Progression of the disease leads to narrowed pulmonary veins, pulmonary hypertension, congestion, and edema (swelling) of the lungs.

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