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 veins in the liver are obstructed. It is a complication of high-dose chemotherapy given before a bone marrow transplant and/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.^[1] The name sinusoidal obstruction syndrome (SOS) is preferred if hepatic veno-occlusive disease happens as a result of chemotherapy or bone marrow transplantation.^[1]^[2]

Apart from chemotherapy, hepatic veno-occlusive disease 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]

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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.^[4]

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 supportive care including pain management and possibly diuretics.^[5] In those with severe disease due to a bone marrow transplant, defibrotide is a proposed treatment.^[6] It has been approved for use in severe cases in Europe and the United States.^[7]^[8] A placebo controlled trial, however, has not been done as of 2016.^[5]

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]

References

^ ^a ^b ^c ^d ^e Helmy A (January 2006). "Review article: updates in the pathogenesis and therapy of hepatic sinusoidal obstruction syndrome (SOS)". Aliment. Pharmacol. Ther. 23 (1): 11–25. doi:10.1111/j.1365-2036.2006.02742.x. PMID 16393276. S2CID 22462806.
^ 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. S2CID 260317941.
^ 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" (PDF). Nat. Genet. 38 (6): 620–2. doi:10.1038/ng1780. PMID 16648851. 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)
^ ^a ^b ^c Dalle, JH; Giralt, SA (March 2016). "Hepatic Veno-Occlusive Disease after Hematopoietic Stem Cell Transplantation: Risk Factors and Stratification, Prophylaxis, and Treatment". Biology of Blood and Marrow Transplantation. 22 (3): 400–9. doi:10.1016/j.bbmt.2015.09.024. PMID 26431626.
^ ^a ^b Fulgenzi, A; Ferrero, ME (2016). "Defibrotide in the treatment of hepatic veno-occlusive disease". Hepatic Medicine: Evidence and Research. 8: 105–113. doi:10.2147/HMER.S79243. PMC 5098529. PMID 27843363.
^ Keating, GM (December 2014). "Defibrotide: a review of its use in severe hepatic veno-occlusive disease following haematopoietic stem cell transplantation". Clinical Drug Investigation. 34 (12): 895–904. doi:10.1007/s40261-014-0242-x. PMID 25351934. 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.
^ Willmot, F; Robertson, George (1920). "Senecio disease, or cirrhosis of the liver due to senecio poisoning". The Lancet. 196 (5069): 848–849. doi:10.1016/S0140-6736(01)00020-4.

External links

Classification	D ICD-10: K76.5 OMIM: 235550 MeSH: D006504 DiseasesDB: 34365 SNOMED CT: 65617004
External resources	eMedicine: ped/2396 GeneReviews: Venous Occlusive Disease with Immunodeficiency Orphanet: 890

Cardiovascular disease (vessels)

Arteries, arterioles
and capillaries

Inflammation

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 human digestive system

Upper GI tract

Esophagus

Stomach

Lower GI tract
Enteropathy

Small intestine (Duodenum/Jejunum/Ileum)	Enteritis Duodenitis Jejunitis Ileitis Peptic (duodenal) ulcer Curling's ulcer Malabsorption: Coeliac Tropical sprue Blind loop syndrome Small intestinal bacterial overgrowth Whipple's Short bowel syndrome Steatorrhea Milroy disease Bile acid malabsorption
Large intestine (Appendix/Colon)	Appendicitis Colitis Pseudomembranous Ulcerative Ischemic Microscopic Collagenous Lymphocytic Dysentery Functional colonic disease IBS Intestinal pseudoobstruction / Ogilvie syndrome Megacolon / Toxic megacolon Diverticulitis/Diverticulosis/SCAD
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 Solitary rectal ulcer syndrome
Anal canal	Anal fissure/Anal fistula Anal abscess Hemorrhoid Anal dysplasia Pruritus ani

GI bleeding

Accessory

Liver	Hepatitis Viral hepatitis Autoimmune hepatitis Alcoholic hepatitis Cirrhosis PBC Fatty liver MASLD 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 Gallstone / Cholelithiasis Cholesterolosis Adenomyomatosis Postcholecystectomy syndrome Porcelain gallbladder
Bile duct/ Other biliary tree	Cholangitis Primary sclerosing cholangitis Secondary sclerosing cholangitis Ascending Cholestasis/Mirizzi's syndrome Biliary fistula Haemobilia Common bile duct Choledocholithiasis Biliary dyskinesia Sphincter of Oddi dysfunction
Pancreatic	Pancreatitis Acute Chronic Hereditary Pancreatic abscess Pancreatic pseudocyst Exocrine pancreatic insufficiency Pancreatic fistula

Other

Hernia	Diaphragmatic Congenital Hiatus Inguinal Indirect Direct Umbilical Femoral Obturator Spigelian Lumbar Petit's Grynfeltt–Lesshaft Undefined location Incisional Internal hernia Richter's
Peritoneal	Peritonitis Spontaneous bacterial peritonitis Hemoperitoneum Pneumoperitoneum

This page was last edited on 30 August 2023, at 15:01

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