[Primary amyloidosis as a rare cause of acute liver insufficiency]

A 67-year-old woman with marked intrahepatic cholestasis died of acute liver failure with multiple organ failure six weeks after the first onset of jaundice. A primary generalized amyloidosis was unexpectedly found at necropsy. It had taken an unusual clinical course due to the terminal loss of liver function. Extensive serological and imaging tests had not provided a uniform diagnosis which could have been made in life only by an early liver biopsy. As icteric amyloidosis of the liver is invariably fatal within a few months, this disease, though rare, should be considered in the differential diagnosis of unclear intrahepatic cholestasis.