Hepatic veno-occlusive disease

Hepatic veno-occlusive disease
Classification and external resources
Specialty	gastroenterology
ICD-10	K76.5
OMIM	235550
DiseasesDB	34365
eMedicine	ped/2396
MeSH	D006504
GeneReviews	Venous Occlusive Disease with Immunodeficiency

Hepatic veno-occlusive disease or veno-occlusive disease (VOD) is a 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 (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 sinusoidal 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]

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.

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.

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.

History

The first report on veno-occlusive disease, in 1920, was as a result of 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]

References

1 2 3 4 5 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.
↑ 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, (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; Robertson, George (1920). "Senecio disease, or cirrhosis of the liver due to senecio poisoning". The Lancet 196 (5069): 848. doi:10.1016/S0140-6736(01)00020-4.

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.

Arteries, arterioles
and capillaries

Inflammation

Arterial occlusive disease /
peripheral artery disease

Arteriosclerosis	Atherosclerosis Foam cell Fatty streak Atheroma Intermittent claudication Critical limb ischemia Monckeberg's arteriosclerosis Arteriolosclerosis Hyaline Hyperplastic Cholesterol LDL Oxycholesterol Trans fat

Stenosis	Carotid artery stenosis Renal artery stenosis

Other	Aortoiliac occlusive disease Degos disease Erythromelalgia Fibromuscular dysplasia Raynaud's phenomenon

Aneurysm / dissection /
pseudoaneurysm

Vascular malformation

Vascular nevus

Veins

Inflammation	Phlebitis

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

Varicose veins	Gastric varices Portacaval anastomosis Caput medusae Esophageal varices Hemorrhoid Varicocele

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

Arteries or veins

Blood pressure

Hypertension	Hypertensive heart disease Hypertensive emergency Hypertensive nephropathy Essential hypertension Secondary hypertension Renovascular hypertension Benign hypertension Pulmonary hypertension Systolic hypertension White coat hypertension

Hypotension	Orthostatic hypotension

Diseases of the digestive system (primarily K20–K93, 530–579)

Upper GI tract

Esophagus	Esophagitis Candidal Eosinophilic Herpetiform Rupture Boerhaave syndrome Mallory-Weiss syndrome UES Zenker's diverticulum LES Barrett's esophagus Esophageal motility disorder Nutcracker esophagus Achalasia Diffuse esophageal spasm Gastroesophageal reflux disease (GERD) Laryngopharyngeal reflux (LPR) Esophageal stricture Megaesophagus

Stomach	Gastritis Atrophic Ménétrier's disease Gastroenteritis Peptic (gastric) ulcer Cushing ulcer Dieulafoy's lesion Dyspepsia Pyloric stenosis Achlorhydria Gastroparesis Gastroptosis Portal hypertensive gastropathy Gastric antral vascular ectasia Gastric dumping syndrome Gastric volvulus

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 Small bowel bacterial overgrowth syndrome Whipple's Short bowel syndrome Steatorrhea Milroy disease bile acid malabsorption

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 Gastroenterocolitis 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 Anismus

Anal canal	Anal fissure/Anal fistula Anal abscess Anal dysplasia Pruritus ani

GI bleeding/BIS

Accessory

Liver	Hepatitis Viral hepatitis Autoimmune hepatitis Alcoholic hepatitis Cirrhosis PBC Fatty liver NASH Vascular Budd-Chiari syndrome 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 Metabolic disorders Wilson's Disease Hemochromatosis

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

Bile duct/ Other biliary tree	Cholangitis Primary sclerosing cholangitis 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 abscess Pancreatic pseudocyst Exocrine pancreatic insufficiency Pancreatic fistula

Abdominopelvic

Hernia	Diaphragmatic Congenital Hiatus Inguinal Indirect Direct Umbilical Femoral Obturator Spigelian Lumbar Petit's Grynfeltt-Lesshaft Undefined location Incisional Internal hernia

Peritoneal	Peritonitis Spontaneous bacterial peritonitis Hemoperitoneum Pneumoperitoneum

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