Severe Intrahepatic Cholestasis in an Elderly Patient with Primary Amyloidosis and Colon Adenocarcinoma

Hepatic involvement in primary amyloidosis (AL type) is not rare but is often clinically silent. However, presentation with jaundice in AL-type amyloidosis is rare, with an incidence of less than 5% reported in the literature. It is considered to be a preterminal sign. We herein report on a case of primary hepatic amyloidosis presenting with severe intrahepatic cholestasis. Viral, drug, alcohol, and autoimmune etiologies were all excluded. A liver biopsy was performed because of unexplained cholestatic jaundice for 3 months. The pathology showed hepatic amyloidosis with extensive amyloid deposition in the expanded portal tracts and sinusoidal space. The patient received supportive treatment only, because of persistent jaundice, coexistent colon cancer with para-aortic lymph node metastasis, and possibly peritoneal carcinomatosis. Unfortunately, the patient died of sepsis 10 months after the onset of jaundice. We suggest that hepatic amyloidosis must be considered in the differential diagnosis of unexplained cholestatic jaundice.