Cholestasis and liver failure with lambda-AL amyloidosis.
Clinically significant liver involvement in systemic amyloidosis, especially with cholestasis, is rare. We report a case of primary amyloidosis with severe intrahepatic cholestasis leading to terminal liver failure. The present case is the first of its kind reported involving Lambda light chains only as the associated paraprotein. Conventional treatment and a therapeutic trial with dimethyl sulphoxide were unsuccessful.
D000368:Aged; D000686:Amyloidosis / Q000150:complications* / Q000276:immunology / Q000473:pathology; D002779:Cholestasis / Q000209:etiology*; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000032:analysis; D007146:Immunoglobulin lambda-Chains / Q000032:analysis; D008107:Liver Diseases / Q000209:etiology* / Q000473:pathology; D008297:Male
D007147:Immunoglobulin Light Chains; D007146:Immunoglobulin lambda-Chains