Primary Amyloidosis Presenting with Nephrotic Syndrome and Atypical Intrahepatic Cholestasis: Report of 2 Cases

Abstract Liver is one of the most commonly involved organs in both primary and secondary systemic amyloidoses, but hepatic amyloidosis, manifested as mild to moderate enlargement, is usually not symptomatic nor it is clinically problematic. Rarely, massive hepatomegaly, severe cholestatic hepatitis or liver failure may be encountered in patients with systemic amyloidosis. Two cases with lambda light-chain amyloidosis presenting with nephrotic syndrome and atypical intrahepatic cholestasis are discussed with clinical features, laboratory and kidney, liver and bone marrow biopsy findings in view of the relevant literature.