primary hepatic amyloidosis with severe cholestasis: a case report

despite the fact that hepatic involvement is frequently seen in systemic primary amyloidosis, major hepatic symptoms as primary manifestation and severe impaired liver function are rare. herein, we report a 38-year-old woman with primary hepatic amyloidosis, and severe  portal hypertension . the patient had ascites and markedly elevated alkaline phosphatase level at presentation. she had a rapid downhill course resulting in death. in case of an unexplained intrahepatic cholestasis or portal hypertension the possibility of amyloidosis should be considered and a  congo red staining should be performed.