False about Budd-Chiari syndrome

Budd-Chiari syndrome in its pure form consists of slowly developing thrombosis of the hepatic veins and the adjacent inferior vena cava . ETIOLOGY.: idiopathic in one third, in the remaining cases various causes associated with increased thrombotic tendencies are attributed to polycythaemia vera, paroxysmal nocturnal haemoglobinuria, oral contraceptives, pregnancy, postpaum state, intra-abdominal cancers (e.g. hepatocellular carcinoma), chemotherapy, radiation and myeloproliferative diseases. Formation of membranous webs, probably congenital or as a consequence of organised thrombosis, in the suprahepatic poion of inferior vena cava is another impoant cause. MORPHOLOGIC FEATURES. Grossly, the liver is enlarged, swollen, red-purple and has a tense capsule. Histologically, the changes in sudden hepatic vein occlusion are those of centrilobular congestion, necrosis and rupture of sinusoids into the space of Disse. In slowly developing thrombosis, the changes are more chronic and include fibrosing reaction in the centrilobular zone that may progress to cardiac cirrhosis. CLINICAL FEATURES : acute form -abdominal pain, vomiting, enlarged liver, ascites and mild icterus. chronic form - the patients present with pain over enlarged tender liver, ascites and other features of poal hypeension. The acute form of illness leads to acute hepatic failure and death, whereas in chronic form the patient may live for months to a few years. TEXT BOOK OF PATHOLOGY 6TH EDITION HARSH MOHAN PGNO.603 IMAGE REF: