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#)

• strong association with acute rejection (mortality: 63%) (#10534334#)

Variants

 fetal hepatic veno-occlusive disease (#12835579#)

• pyrrolizidine alkaloids of food origin

Localization

 hepatic veno-occlusive disease

• liver allograft veno-occlusive disease

 pulmonary veno-occlusive disease

See also

 veno-occlusive lesions
 veno-occlusive diseases