A case of prominent hepatic cholestasis developing to hepatic failure in lambda-AL amyloidosis.
We report a case of lambda-AL amyloidosis which manifested prominent hepatic cholestasis. The patient was a 71-year-old Japanese male who was admitted to our hospital because of abdominal distension and jaundice. Laboratory examination revealed a marked deterioration of liver function with cholestasis. Gastric biopsy revealed amyloid deposition. Under a diagnosis of primary amyloidosis he was treated with corticosteroid and dimethylsulfoxide. However, jaundice progressed, renal function deteriorated rapidly, and he died two weeks after admission. Autopsy revealed a profound deposition of lambda-AL amyloid not only in the liver but also in the kidneys, spleen, lungs, heart and intestine.
Dohmen
K
K
Department of Internal Medicine, Kyushu Koseinenkin Hospital, Kitakyushu, Japan.
Nagano
M
M
Iwakiri
R
R
Yamano
Y
Y
Kikuchi
Y
Y
Mizoguchi
M
M
Iwata
Y
Y
Mori
Y
Y
Ishibashi
H
H
D000368:Aged; D000682:Amyloid / Q000378:metabolism*; D000686:Amyloidosis / Q000150:complications* / Q000473:pathology; D002780:Cholestasis, Intrahepatic / Q000175:diagnosis / Q000209:etiology* / Q000473:pathology; D006801:Humans; D008099:Liver / Q000378:metabolism* / Q000473:pathology; D008111:Liver Function Tests; D008297:Male; D012720:Severity of Illness Index
D000682:Amyloid; C035222:amyloid protein AL