Veno-occlusive disease cirrhosis

Hepatic Effects. Severe cirrhosis of the liver was one of the primary systemic effects seen following injection of Thorotrast in humans (Baxter et al. 1980a, b Faber 1979 Kato and Kido 1987 Kato et al. 1983 Mori et al. 1979, 1983a, b Rao et al. 1986 Van Kaick et al. 1983). Cases of fibrosis, veno-occlusive disease, and blood-filled cavities were also found in the livers of Thorotrast patients (da Silva Horta 1967a Dejgaard et al. 1984). The latency period for the appearance of the cirrhosis was not clear, but was probably comparable to the latency period for liver tumors (25-30 years) since the two effects were often found together. 

Occasionally toxic compounds can directly damage the hepatic sinusoids and capillaries. One such toxic compound is monocrotaline, a naturally occurring pyrrolozidine alkaloid, found in certain plants (Heliotropium, Senecio, and Crotolaria species). Monocrotaline (Fig. 7.7) is metabolized to a reactive metabolite, which is directly cytotoxic to the sinusoidal and endothelial cells, causing damage and occlusion of the lumen. The blood flow in the liver is therefore reduced and ischemic damage to the hepatocytes ensues. Centrilobular necrosis results, and the venous return to the liver is blocked. Hence, this is known as veno-occlusive disease and results in extensive alteration in hepatic vasculature and function. Chronic exposure causes cirrhosis. 

Veno-occlusive disease (VOD) differs from Budd-Chiari syndrome in that it consists of occlusive fibrosis of the small intrahepatic veins. VOD may present as either an acute form with sudden ascites, liver enlargement and rapidly rising bilirubin, or as a chronic form with fibrosis and cirrhosis. One of the main causes of VOD is the use of cyclophosphamide or alkalating agents during conditioning for bone marrow transplantation, where it occurs in up to 20% of cases. Other causes include irradiation, antineoplastic drugs, pyrrolizidine alkaloids and alcohol. 

Bras, G., Jelliffe, D.B., Stu, K.L. Veno-occlusive disease of the liver with non-portal type of cirrhosis occurring in Jamaica. Arch. Pathol. 

An 18-month-old boy who had regularly consumed a herbal tea mixture since the 3rd month of hfe developed veno-occlusive disease with portal hypertension and severe ascites (46). Histology of the liver showed centrilobular sinusoidal congestion with perivenular bleeding and parenchymal necrosis without cirrhosis. The child was given conservative treatment only and recovered completely within 2 months. 

The main type of Uver damage caused by S. officinale is veno-occlusive disease, a non-thrombotic obliteration of small hepatic veins leading to cirrhosis and eventually liver failure (3). Patients can present with acute or chronic signs portal hypertension, hepatomegaly, and abdominal pain are the main features. 

In a South African study 20 children were identified as suffering from hepatic veno-occlusive disease thought to be caused by the administration of traditional remedies (3). The predominant clinical presentation was ascites and hepatomegaly. Nine children died. The surviving patients progressed to cirrhosis and portal hypertension. In four cases early urine specimens were available, and in all of these the presence of pyrrolizidine alkaloids was confirmed. 

While some of these alkaloids have shown carcinogenic and mutagenic properties, and kidney toxicity has been reported (Fu et al. 2004), the primary concern for use of these herbs is the potential for serious liver damage, specifically hepatic veno-occlusive disease (a condition in which veins in the liver become blocked). This potentially fatal condition manifests symptoms such as abdominal pain, swelling of the liver and spleen, accumulation of fluid in the abdominal cavity, elevated levels of bilirubin, jaundice, cirrhosis of the liver, and liver failure (Chen and Huo 2010 McDermott and Ridker 1990). 

Bras G, Jelliffe DB, Stuart KL (1954) Veno-occlusive disease in liver with nonportal type of cirrhosis, occurring in Jamaica. Arch Path 57 285-300... 

Many pyrrolizidine alkaloids are hepato-toxic, mutagenic, and carcinogenic. They can cause veno-occlusive disease of the liver that may lead to cirrhosis and eventually liver failure. The main reasons for intoxications with pyrrolizidines are contamination of cereals with... 

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