[Massive hepatic amyloidosis: a case of hepatic failure and haemorrhagic pancreatitis of fatal evolution].

Hepatic involvement is frequently in systemic amyloidosis but major clinical symptoms due to portal hypertension or liver failure are rare. To date all treatment modalities proven in these patients have failed. Thus, prognosis is dismal with progressive deterioration in liver function. We describe a patient with massive liver involvement by primary amyloidosis, manifested by severe intrahepatic cholestasis. Up to now 25 similar case have been reported in the English literature. In this subset of patients the most frequently recorded cause of death was renal failure accelerated by hyperbilirrubunemia. In our patient a downhill course was characterized by fatal renal hepatic failure after an hemoperitoneum, probably as a delayed complication of liver biopsy. Although not all authors agree, an added risk of bleeding after liver biopsy have been pointed out in hepatic amyloidosis. This kind of problem recommends the use of tissue other than liver, or a transjugular hepatic biopsy for diagnostic purposes. Also, of interest in the present cause is the autopsy findings of pancreatitis associated to pancreatic amyloidosis, a complication which have been described in previous reports.