Hepatomegaly and cholestasis as primary clinical manifestations of an AL-kappa amyloidosis.
A 53-year-old man, who presented with weight loss over a period of 10 months, hepatomegaly, markedly raised cholestatic enzymes and Ca 19-9, was initially suspected of suffering from metastatic cholangio-carcinoma. Liver biopsy revealed depositions of AL-amyloid. Further investigations confirmed a generalized amyloidosis. Biopsies taken from the gastric, colonic, and bronchial mucosa all showed depositions of amyloid. A nephrotic syndrome was interpreted as being secondary to the renal involvement. Echocardiography identified changes which were consistent with cardiac involvement. A plasmacytoma or lymphoma was excluded. At the time of diagnosis the patient was in a good physical condition with normal renal function. Within a few weeks the renal function deteriorated and after 2 months the patient developed ascites and became jaundiced. Four months after initial presentation the patient died from cardiac failure.
Mohr
A
A
Department of Gastroenterology, Hepatology and Infectious Diseases, University of Magdeburg, Germany.
Miehlke
S
S
Klauck
S
S
Röcken
C
C
Malfertheiner
P
P
D000682:Amyloid / Q000378:metabolism; D000686:Amyloidosis / Q000097:blood / Q000175:diagnosis* / Q000378:metabolism / Q000473:pathology; D001706:Biopsy; D002758:Cholangiography; D002779:Cholestasis / Q000175:diagnosis*; D017809:Fatal Outcome; D006529:Hepatomegaly / Q000175:diagnosis*; D006801:Humans; D007145:Immunoglobulin kappa-Chains / Q000378:metabolism; D008099:Liver / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology; D008297:Male; D008875:Middle Aged
D000682:Amyloid; D007145:Immunoglobulin kappa-Chains