Hepatic veno-occlusive disease
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
Apart from chemotherapy, hepatic veno-occlusive disease may also occur after ingestion of certain plant
Signs and symptoms
Features of hepatic veno-occlusive disease include weight gain, tender
Pathophysiology
In the bone marrow transplant setting, hepatic veno-occlusive disease is felt to be due to injury to the hepatic venous endothelium from the conditioning regimen. Toxic agents causing veno-occlusive disease include plants as well as the medication cyclophosphamide.[citation needed]
Diagnosis
Doppler ultrasound of the liver is typically utilized to confirm or suggest the diagnosis. Common findings on liver doppler ultrasound include increased phasicity of portal veins with eventual development of portal flow reversal. The liver is usually enlarged but maintained normal echogenicity. A liver biopsy is required for a definitive diagnosis.[citation needed]
Treatment
Treatment generally includes
Prognosis
Mild disease has a risk of death of about 10% while moderate disease has a risk of death of 20%.[5] When it occurs as a result of bone marrow transplant and multiorgan failure is present, the risk of death is greater than 80%.[6]
History
The first report on veno-occlusive disease, in 1920, was as a result of senecio poisoning in South Africa.[9] Subsequent reports were mostly in Jamaicans who had consumed herbal teas.[1] With the advent of bone marrow transplantation, most cases since its introduction have been in those undergoing treatment for leukemia.[1]
See also
- hepatic vein thrombosis)
References
- ^ S2CID 22462806.
- S2CID 260317941.
- S2CID 1466106.
- ^ RESERVED, INSERM US14 -- ALL RIGHTS. "Orphanet: Hepatic veno occlusive disease". www.orpha.net. Retrieved 2016-12-26.
{{cite web}}
: CS1 maint: numeric names: authors list (link) - ^ PMID 26431626.
- ^ PMID 27843363.
- S2CID 21019656.
- ^ "Press Announcements - FDA approves first treatment for rare disease in patients who receive stem cell transplant from blood or bone marrow". www.fda.gov. Retrieved 18 July 2017.
- .
Further reading
- Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. (2005). Robbins and Cotran pathologic basis of disease. St. Louis, Mo: Elsevier Saunders. ISBN 978-0-7216-0187-8.
- Wingard JR, Nichols WG, McDonald GB (2004). "Supportive care". Hematology Am Soc Hematol Educ Program. 1: 372–89. PMID 15561693.
- Dignan, Fiona L.; Wynn, Robert F.; Hadzic, Nedim; Karani, John; Quaglia, Alberto; Pagliuca, Antonio; Veys, Paul; Potter, Michael N. (1 November 2013). "BCSH/BSBMT guideline: diagnosis and management of veno-occlusive disease (sinusoidal obstruction syndrome) following haematopoietic stem cell transplantation". British Journal of Haematology. 163 (4): 444–457. PMID 24102514.