Home > D. Systemic pathology > Vascular anomalies > veno-occlusive diseases

veno-occlusive diseases

Friday 12 March 2004

Localization

hepatic veino-occlusive disease (hepatic VOD)
pulmonary veino-occlusive disease (pulmonary VOD)

This disease consists of occlusive fibrosis of small intrahepatc veins: centrilobular and sublobular venules. Lager veins and those affected by Budd-Chiari syndrome are spared.

Synopsis

Early lesions

subendothelial edema
centrolobular congestion of the pericentral sinusoids
blockage of blood flow in the pericentral sinusoids by fibrin and platelet aggregates
pericentral sinusoids plugged with fibrin, fibrin deposition
sinusoidal dilatation
centrilobular hemorrhagic necrosis

Late

intraluminal fibrosis
concentric intimal thickening
occlusive fibrosis of small intrahepatic veins (centrolobular veins and sublobular venules), fibrous obliteration of hepatic veins
concentric occlusion of the affected veins by loose connective tissue
fibrotic scar replacing the centrilobular vein
centrolobular fibrosis
centro-portal bridging
cirrhosis

Etiology

conditioning for bone marrow transplantation (onset of the disorder : 3-4 weeks after transplantation; 20% of cases; mortality rate: 50%)

cyclophosphamide
alkalating agents with or without total body irradiation

irradiation (10 Gy of irradiation with chemotherapy; 30Gy without chemotherapy)

antineoplastic drugs

azathioprine (may cause the lesion many months after continuous administration)
6-mercaptopurine
6-thioguanine
mitomycin C
BCNU(carmustine)
vincristine
adriamycin
gemtuzumab

pyrrolizidine alkaloids (found in about 300 species of plants such Heliotropium , Crotolaria and Senecio found all over the world and used to make herbal teas and enemas)

alcoholic liver
congenital immunodeficiency
dimethylnitrosamine
aflatoxin
liver allograft (1.9%) (12640317, 11602848, 8276346, 10534334)