title: κ I light chain AL amyloidosis presenting with rapidly progressive renal and hepatic failure with unusual renal amyloid distribution.
abstract: A 65-year-old man suffering from generalized edema and jaundice was admitted to our hospital. Laboratory findings revealed marked renal dysfunction with heavy proteinuria as well as liver dysfunction with severe obstructive jaundice. On renal biopsy, the diagnosis of AL amyloidosis associated with κ I light chain was made. Interestingly, amyloid deposits were restricted to the glomeruli. Although hemodialysis was initiated, the patient died due to further deterioration of hepatic function. On autopsy, severe intrahepatic cholestasis was observed, and there was marked deposition of AL amyloid in the liver. Literature reviews showed that rapidly progressive renal failure is common in AL amyloidosis patients who presented with acute hepatic failure due to severe intrahepatic cholestasis. However, the detailed renal pathology in this condition has not been documented. The present case is very interesting because rapidly progressive renal and hepatic failure was simultaneously observed, and renal amyloid deposition was restricted to the glomeruli.
authors.affiliation: Department of Nephrology and Pathology, Iwate Prefectural Central Hospital, Morioka, Japan. oaugpkd40@hotmail.com
authors.forename: Yuji
authors.initials: Y
authors.lastname: Oe
authors.forename: Izaya
authors.initials: I
authors.lastname: Nakaya
authors.forename: Mayumi
authors.initials: M
authors.lastname: Yahata
authors.forename: Okinora
authors.initials: O
authors.lastname: Murata
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Yaegashi
authors.forename: Tsutomu
authors.initials: T
authors.lastname: Sakuma
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Sato
authors.forename: Juris J
authors.initials: JJ
authors.lastname: Liepnieks
authors.forename: Merrill D
authors.initials: MD
authors.lastname: Benson
authors.forename: Jun
authors.initials: J
authors.lastname: Soma
mesh_terms: D058186:Acute Kidney Injury / Q000209:etiology / Q000473:pathology*; D000368:Aged; D000595:Amino Acid Sequence; D000686:Amyloidosis / Q000150:complications / Q000235:genetics / Q000473:pathology*; D002780:Cholestasis, Intrahepatic / Q000209:etiology / Q000473:pathology; D018450:Disease Progression; D017809:Fatal Outcome; D006801:Humans; D007145:Immunoglobulin kappa-Chains / Q000235:genetics* / Q000378:metabolism; D007678:Kidney Glomerulus / Q000378:metabolism / Q000473:pathology; D017114:Liver Failure, Acute / Q000209:etiology / Q000473:pathology*; D008297:Male; D008969:Molecular Sequence Data
chemical_list: D007145:Immunoglobulin kappa-Chains