| Hepatic veno-occlusive disease | |
|---|---|
| Other names | Veno-occlusive disease with immunodeficiency Sinusoidal obstruction syndrome |
| Specialty | Gastroenterology  |
| Symptoms | Weight gain, tender enlargement of the liver, ascites, jaundice |
| Diagnostic method | Liver biopsy |
| Differential diagnosis | Budd–Chiari syndrome |
| Prevention | Ursodeoxycholic acid |
| Treatment | Defibrotide |
| Deaths | 10-20% |
Hepatic veno-occlusive disease (VOD) or veno-occlusive disease with immunodeficiency is a potentially life-threatening condition in which some of the small veins in the liver are obstructed. It is a complication of high-dose chemotherapy given before a bone marrow transplant and/or excessive exposure to hepatotoxic pyrrolizidine alkaloids. It is classically marked by weight gain due to fluid retention, increased liver size, and raised levels of bilirubin in the blood.[1] The name sinusoidal obstruction syndrome (SOS) is preferred if hepatic veno-occlusive disease happens as a result of chemotherapy or bone marrow transplantation.[1][2]
Apart from chemotherapy, hepatic veno-occlusive disease may also occur after ingestion of certain plant alkaloids such as pyrrolizidine alkaloids (in some herbal teas),[1] and has been described as part of a rare hereditary disease called hepatic venoocclusive disease with immunodeficiency (which results from mutations in the gene coding for a protein called SP110).[3]
- ^ a b c Helmy A (January 2006). "Review article: updates in the pathogenesis and therapy of hepatic sinusoidal obstruction syndrome (SOS)". Aliment. Pharmacol. Ther. 23 (1): 11–25. doi:10.1111/j.1365-2036.2006.02742.x. PMID 16393276. S2CID 22462806.
- ^ DeLeve LD, Shulman HM, McDonald GB (February 2002). "Toxic injury to hepatic sinusoids: sinusoidal obstruction syndrome (veno-occlusive disease)". Semin. Liver Dis. 22 (1): 27–42. doi:10.1055/s-2002-23204. PMID 11928077. S2CID 260317941.
- ^ Roscioli T, Cliffe ST, Bloch DB (June 2006). "Mutations in the gene encoding the PML nuclear body protein Sp110 are associated with immunodeficiency and hepatic veno-occlusive disease" (PDF). Nat. Genet. 38 (6): 620–2. doi:10.1038/ng1780. PMID 16648851. S2CID 1466106.