Hepatic Complications in Polycythaemia Vera

Veno-occlusive Disease of Liver

“…recently recognized as a separate entity. The disease is seen predominantly in children, but it has also been reported in adults (Stuart and Bras, 1957). In the acute stage the small hepatic veins are involved and to a less degree the medium-sized vessels.…”

“…A number, however, die in the acute stage from liver failure, while others pass into a subacute or chronic stage. The chronic phase is characterized by a picture of cirrhosis of the liver which is indistinguishable from that of other forms of cirrhosis (Stuart and Bras, 1957).…”

“…In the acute stage the diagnosis is usually based on the history, the characteristic clinical picture, and the liverbiopsy findings (Stuart and Bras, 1957).…”

“…The former is known to be the essenrial pathological basis of Budd-Chiari syndrome, and the latter of the " veno-occlusive disease of the liver" reported from Jamaica and other places (Jelliffe et al, 1954;Bras and Hill, 1956). In Budd-Chiari syndrome the obstruction in the main hepatic vein or veins occurs mostly secondarily to other disease processes such as pressure and invasion from adjoining hepatic neoplasms, vena-caval spread of renal carcinoma, thrombophlebitis migrans, portal pyaemia (Chiari, 1899), and polycythaemic states (Sohval, 1938). In many cases, however, no such cause or association can be found, and Chiari (1899) favoured primary inflammation of one or more hepatic veins, leading to thrombosis, as a cause for such cases.…”

“…The entity described as veno-occlusive disease of the liver from Jamaica and other places differs from Budd-Chiari syndrome in that the cause is ingestion of toxic weeds and other substances, and the essential pathology is an obliterative endophlebitis of the small tributaries of the hepatic venous system (Stuart and Bras, 1957).…”