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Wikipedia - Hepatic veno-occlusive disease

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Hepatic veno-occlusive disease
Classification and external resources
ICD-10	K 76.5
OMIM	235550
DiseasesDB	34365
eMedicine	ped/2396
MeSH	D006504

Hepatic veno-occlusive disease or veno-occlusive disease (VOD) is a condition in which some of the small veins in the liver are blocked. It is a complication of high-dose chemotherapy given before a bone marrow transplant (BMT) and is marked by weight gain due to fluid retention, increased liver size, and raised levels of bilirubin in the blood.^[1] The name sinuosoidal obstruction syndrome is now preferred if VOD happens as a result of chemotherapy or bone marrow transplantation.^[1]^[2]

Apart from chemotherapy, VOD 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]

1 Signs and symptoms
2 Diagnosis
3 Pathophysiology
4 Treatment
5 Prognosis
6 History
7 See also
8 References
9 External links
10 Further reading

[edit] Signs and symptoms

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

[edit] 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.

[edit] Pathophysiology

In the BMT setting, VOD is felt to be due to injury to the hepatic venous endothelium from the conditioning regimen.

[edit] 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 developoment of defibrotide for this indication is uncertain.

[edit] 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.

[edit] History

The first report on veno-occlusive disease, in 1920, was as a result senecio poisoning in South Africa.^[4] Subsequent reports were mostly in Jamaicans who had consumed herbal teas.^[1] With the advent of bone marrow transplanation, most later reported cases have been in those undergoing treatment for leukemia.^[1]

[edit] See also

Budd-Chiari syndrome (liver vein obstruction due to thrombosis)

[edit] References

^ ^a ^b ^c ^d ^e Helmy A (January 2006). "Review article: updates in the pathogenesis and therapy of hepatic sinusoidal obstruction syndrome". Aliment. Pharmacol. Ther. 23 (1): 11–25. doi:10.1111/j.1365-2036.2006.02742.x. PMID 16393276. http://www3.interscience.wiley.com/cgi-bin/fulltext/118572108/HTMLSTART.
^ 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.
^ Roscioli T, Cliffe ST, Bloch DB, et al. (June 2006). "Mutations in the gene encoding the PML nuclear body protein Sp110 are associated with immunodeficiency and hepatic veno-occlusive disease". Nat. Genet. 38 (6): 620–2. doi:10.1038/ng1780. PMID 16648851.
^ Willmot, F (1920). "Senecio Disease, Or Cirrhosis Of The Liver Due To Senecio Poisoning". The Lancet 196: 848. doi:10.1016/S0140-6736(01)00020-4.

[edit] External links

GeneReview/NIH/UW entry on Hepatic Veno-Occlusive Disease with Immunodeficiency

[edit] 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 0-7216-0187-1.
Wingard JR, Nichols WG, McDonald GB (2004). "Supportive care". Hematology Am Soc Hematol Educ Program 1: 372–89. doi:10.1182/asheducation-2004.1.372. PMID 15561693. http://asheducationbook.hematologylibrary.org/cgi/content/full/2004/1/372.

Cardiovascular disease: vascular disease · Circulatory system pathology (I70–I99, 440–456)

Arteries, arterioles
and capillaries

Inflammation

Arteritis (Aortitis) · Buerger's disease

Arterial occlusive disease/
peripheral vascular disease

Aneurysm/dissection/
pseudoaneurysm

torso: Aortic aneurysm (Thoracic aortic aneurysm, Abdominal aortic aneurysm) · Aortic dissection · Coronary artery aneurysm

head/neck: Cerebral aneurysm · Intracranial berry aneurysm · Carotid artery dissection · Vertebral artery dissection · Familial aortic dissection

Vascular malformation

Arteriovenous fistula · Telangiectasia (Hereditary hemorrhagic telangiectasia)

Vascular nevus

Spider angioma · Halo nevus · Cherry hemangioma

Veins

Inflammation	Phlebitis

Venous thrombosis/ Thrombophlebitis	primarily lower limb (Deep vein thrombosis) abdomen (May-Thurner syndrome, Portal vein thrombosis, Budd-Chiari syndrome, Renal vein thrombosis) upper limb/torso (Paget-Schroetter disease, Mondor's disease) head (Cerebral venous sinus thrombosis) Post-thrombotic syndrome

Varicose veins	Varicocele · Gastric varices · Portacaval anastomosis (Hemorrhoid, Esophageal varices, Caput medusae)

Other	Superior vena cava syndrome · Inferior vena cava syndrome · Venous ulcer · Chronic venous insufficiency · Chronic cerebrospinal venous insufficiency

Arteries or veins

Vasculitis · Thrombosis · Embolism (Pulmonary embolism, Cholesterol embolism, Paradoxical embolism) · Angiopathy (Macroangiopathy, Microangiopathy)

Blood pressure

Hypertension	Hypertensive heart disease · Hypertensive nephropathy · Essential hypertension · Secondary hypertension (Renovascular hypertension) · Pulmonary hypertension · Malignant hypertension · Benign hypertension · Systolic hypertension · White coat hypertension

Hypotension	Orthostatic hypotension

M: VAS

anat(a:h,u,t,a,l,v:h,u,t,a,l)/phys/devp

noco/syva/cong/tumr, sysi/epon

proc, drug(C3,C4,C5,C9)

Digestive system · Digestive disease · Gastroenterology (primarily K20–K93, 530–579)

Upper GI tract

Lower GI tract:
Intestinal/
enteropathy

Small intestine/ (duodenum/jejunum/ileum)	Enteritis (Duodenitis, Jejunitis, Ileitis) — Peptic (duodenal) ulcer (Curling's ulcer) — Malabsorption: Coeliac · Tropical sprue · Blind loop syndrome · Whipple's · Short bowel syndrome · Steatorrhea · Milroy disease

Large intestine (appendix/colon)	Appendicitis · Colitis (Pseudomembranous, Ulcerative, Ischemic, Microscopic, Collagenous, Lymphocytic) · Functional colonic disease (IBS, Intestinal pseudoobstruction/Ogilvie syndrome) — Megacolon/Toxic megacolon · Diverticulitis/Diverticulosis

Large and/or small	Enterocolitis (Necrotizing) · IBD (Crohn's disease) — vascular: Abdominal angina · Mesenteric ischemia · Angiodysplasia — Bowel obstruction: Ileus · Intussusception · Volvulus · Fecal impaction — Constipation · Diarrhea (Infectious) · Intestinal adhesions

Rectum	Proctitis (Radiation proctitis) · Proctalgia fugax · Rectal prolapse

Anus	Anal fissure/Anal fistula · Anal abscess · Anal dysplasia · Pruritus ani

GI bleeding/BIS

Upper (Hematemesis, Melena) · Lower (Hematochezia)

Accessory

Liver	Hepatitis (Viral hepatitis, Autoimmune hepatitis, Alcoholic hepatitis) · Cirrhosis (PBC) · Fatty liver (NASH) · vascular (Hepatic veno-occlusive disease, Portal hypertension, Nutmeg liver) · Alcoholic liver disease · Liver failure (Hepatic encephalopathy, Acute liver failure) · Liver abscess (Pyogenic, Amoebic) · Hepatorenal syndrome · Peliosis hepatis

Gallbladder	Cholecystitis · Gallstones/Cholecystolithiasis · Cholesterolosis · Rokitansky-Aschoff sinuses · Postcholecystectomy syndrome · Porcelain gallbladder

Bile duct/ other biliary tree	Cholangitis (PSC, Secondary sclerosing cholangitis, Ascending) · Cholestasis/Mirizzi's syndrome · Biliary fistula · Haemobilia · Gallstones/Cholelithiasis common bile duct (Choledocholithiasis, Biliary dyskinesia) · Sphincter of Oddi dysfunction

Pancreatic	Pancreatitis (Acute, Chronic, Hereditary) · Pancreatic pseudocyst · Exocrine pancreatic insufficiency · Pancreatic fistula

Abdominopelvic

Hernia	Diaphragmatic: Congenital diaphragmatic · Hiatus — Abdominal hernia: Inguinal (Indirect, Direct) · Umbilical · Incisional · Femoral — Obturator hernia · Spigelian hernia · Internal hernia

Peritoneal	Peritonitis (Spontaneous bacterial peritonitis) · Hemoperitoneum · Pneumoperitoneum