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Hepatic veno-occlusive disease

Obstruction of small blood vessels in the liver

Field	Value
name	Hepatic veno-occlusive disease
synonyms	Veno-occlusive disease with immunodeficiency
Sinusoidal obstruction syndrome
symptoms	Weight gain, tender enlargement of the liver, ascites, jaundice
diagnosis	Liver biopsy
differential	Budd–Chiari syndrome
prevention	Ursodeoxycholic acid
treatment	Defibrotide
deaths	10-20%

Sinusoidal obstruction syndrome 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 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. The name sinusoidal obstruction syndrome (SOS) is preferred if hepatic veno-occlusive disease happens as a result of chemotherapy or bone marrow transplantation.

Apart from chemotherapy, hepatic veno-occlusive disease may also occur after ingestion of certain plant alkaloids such as pyrrolizidine alkaloids (in some herbal teas),

Signs and symptoms

Features of hepatic veno-occlusive disease include weight gain, tender enlargement of the liver, ascites, and yellow discoloration of the skin; it often is associated with acute kidney failure.

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.

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.

Treatment

Treatment generally includes supportive care including pain management and possibly diuretics. In those with severe disease due to a bone marrow transplant, defibrotide is a proposed treatment. A placebo controlled trial, however, has not been done as of 2016.

Prognosis

Mild disease has a risk of death of about 10% while moderate disease has a risk of death of 20%.

History

The first report on veno-occlusive disease, in 1920, was as a result of senecio poisoning in South Africa. Subsequent reports were mostly in Jamaicans who had consumed herbal teas. With the advent of bone marrow transplantation, most cases since its introduction have been in those undergoing treatment for leukemia.

References

Helmy A. (January 2006). "Review article: updates in the pathogenesis and therapy of hepatic sinusoidal obstruction syndrome (SOS)". Aliment. Pharmacol. Ther..
(February 2002). "Toxic injury to hepatic sinusoids: sinusoidal obstruction syndrome (veno-occlusive disease)". Semin. Liver Dis..
RESERVED, INSERM US14 -- ALL RIGHTS. "Orphanet: Hepatic veno occlusive disease".
(March 2016). "Hepatic Veno-Occlusive Disease after Hematopoietic Stem Cell Transplantation: Risk Factors and Stratification, Prophylaxis, and Treatment.". Biology of Blood and Marrow Transplantation.
(December 2014). "Defibrotide: a review of its use in severe hepatic veno-occlusive disease following haematopoietic stem cell transplantation.". Clinical Drug Investigation.
"Press Announcements - FDA approves first treatment for rare disease in patients who receive stem cell transplant from blood or bone marrow".
(2016). "Defibrotide in the treatment of hepatic veno-occlusive disease.". Hepatic Medicine: Evidence and Research.
Willmot, F. (1920). "Senecio disease, or cirrhosis of the liver due to senecio poisoning". The Lancet.

Info: Wikipedia Source

This article was imported from Wikipedia and is available under the Creative Commons Attribution-ShareAlike 4.0 License. Content has been adapted to SurfDoc format. Original contributors can be found on the article history page.

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