View source for Hepatic veno-occlusive disease

{{SI}}
{{Infobox disease
 | Name            = Hepatic veno-occlusive disease
 | Image           =
 | Caption         =
 | DiseasesDB      = 34365
 | ICD10           = {{ICD10|K|76|5|k|70}}
 | ICD9            =
 | ICDO            =
 | OMIM            = 235550
 | MedlinePlus     =
 | eMedicineSubj   = ped
 | eMedicineTopic  = 2396
 | MeshID          = D006504
 | GeneReviewsNBK   = NBK1271
 | GeneReviewsName = Venous Occlusive Disease with Immunodeficiency
}}
'''Hepatic veno-occlusive disease''' or '''veno-occlusive disease''' ('''VOD''') is a condition in which some of the small [[vein]]s in the [[liver]] are obstructed. It is a [[complication (medicine)|complication]] of high-dose [[chemotherapy]] given before a [[bone marrow transplant]] (BMT) and is marked by weight gain due to [[Water retention (medicine)|fluid retention]], [[hepatomegaly|increased liver size]], and raised levels of [[bilirubin]] in the blood.<ref name=Helmy>{{cite journal |author=Helmy A |title=Review article: updates in the pathogenesis and therapy of hepatic sinusoidal obstruction syndrome |journal=Aliment. Pharmacol. Ther. |volume=23 |issue=1 |pages=11–25 |year=2006 |month=January |pmid=16393276 |doi=10.1111/j.1365-2036.2006.02742.x |url=http://www3.interscience.wiley.com/cgi-bin/fulltext/118572108/HTMLSTART}}</ref> The name '''sinusoidal obstruction syndrome''' is now preferred if VOD happens as a result of chemotherapy or bone marrow transplantation.<ref name=Helmy/><ref>{{cite journal |author=DeLeve LD, Shulman HM, McDonald GB |title=Toxic injury to hepatic sinusoids: sinusoidal obstruction syndrome (veno-occlusive disease) |journal=Semin. Liver Dis. |volume=22 |issue=1 |pages=27–42 |year=2002 |month=February |pmid=11928077 |doi=10.1055/s-2002-23204}}</ref>

Apart from chemotherapy, VOD may also occur after ingestion of certain plant [[alkaloids]] such as [[pyrrolizidine alkaloids]] (in some herbal teas),<ref name=Helmy/> 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]]).<ref>{{cite journal |author=Roscioli T, Cliffe ST, Bloch DB, |title=Mutations in the gene encoding the PML nuclear body protein Sp110 are associated with immunodeficiency and hepatic veno-occlusive disease |journal=Nat. Genet. |volume=38 |issue=6 |pages=620–2 |year=2006 |month=June |pmid=16648851 |doi=10.1038/ng1780 |display-authors=1}}</ref>

==History==
The first report on veno-occlusive disease, in 1920, was as a result [[senecio]] poisoning in [[South Africa]].<ref>{{cite journal | doi=10.1016/S0140-6736(01)00020-4 | title=Senecio Disease, Or Cirrhosis Of The Liver Due To Senecio Poisoning | year=1920 | author=Willmot, F | journal=The Lancet | volume=196 | page=848 | last2=Robertson | first2=Georgew. | issue=5069}}</ref> Subsequent reports were mostly in Jamaicans who had consumed herbal teas.<ref name=Helmy/> With the advent of bone marrow transplanation, most later reported cases have been in those undergoing treatment for leukemia.<ref name=Helmy/>

==Pathophysiology==
In the BMT setting, VOD 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.

==Signs and symptoms==
Features of VOD include weight gain, tender [[hepatomegaly]], [[ascites]], and increased [[bilirubin]].  It often is associated with [[renal failure]].

==Diagnosis==
Hepatic doppler ultrasound is typically utilized to confirm or suggest the diagnosis.  Most 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 for VOD is primarily supportive. In the BMT setting, [[defibrotide]] is an investigational treatment that may be promising. Defibrotide is a polydeoxyribonucleotide isolated from pig intestine. Although its mechanism of action in VOD is unclear, the drug is believed to have antithrombotic properties. In August 2009, Gentium S.p.A., which sponsored the phase 3 clinical trial (pivotal) of defibrotide in hepatic VOD, announced disappointing results. Further clinical development of defibrotide for this indication is uncertain.

==Prognosis==
When associated with bone marrow transplant, VOD is fatal in over 30% of cases. Cases due to plant alkaloids often have a longer and more unpredictable course.

==See also==
* [[Budd-Chiari syndrome]] (large liver vein obstruction due to thrombosis)

==References==
{{reflist|2}}

==Further reading==
* {{cite book |author=Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. |title=Robbins and Cotran pathologic basis of disease |publisher=Elsevier Saunders |location=St. Louis, Mo |year=2005 |pages= |isbn=0-7216-0187-1}}
* {{cite journal |author=Wingard JR, Nichols WG, McDonald GB |title=Supportive care |journal=Hematology Am Soc Hematol Educ Program |pages=372–89 |year=2004 |pmid=15561693 |doi=10.1182/asheducation-2004.1.372 |url=http://asheducationbook.hematologylibrary.org/cgi/content/full/2004/1/372 | volume=1}}

{{Vascular diseases}}
{{Gastroenterology}}

{{DEFAULTSORT:Hepatic Veno-Occlusive Disease}}
[[Category:Hepatology]]
[[Category:Blood disorders]]
[[Category:Rare diseases]]

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