[An unusual cause of hepatorenal symptoms].
METHODS
A 65-year-old patient with normal blood pressure had an exclusive elevation of the cholestasis enzymes (alkaline phosphatase 297 U/l, gamma-GT 315 U/l) and elevated bilirubin levels (1.4 mg/dl) since August 1994. A biopsy of the liver in March 1995 showed features of a "subacute viral hepatitis"; DD drug-induced or toxic lesions. Serological tests gave no support for an acute hepatitis. Intra- or extrahepatic cholestasis could not be proved neither by ultrasound nor by an endoscopic retrograde cholangiopancreatography. Since November 1995 serum creatinine increased up to 1.7 mg/dl (March 1995 1.1 mg/dl) and proteinuria (2.1 g/d) developed. Due to worsening of renal function (serum creatinine 2.8 mg/dl) and increasing proteinuria (3.5 g/d) without nephrotic syndrome, a kidney biopsy was performed. Histologically an amyloidosis (type A lambda) was proven, involving glomerula, kidney vessels and tubules. Further biopsies from the stomach and the duodenum showed profound infiltration of the mucosa and submucosa with amyloid. Therefore, staining of the liver biopsy of March 1995 with congo red proved the diagnosis of liver amyloidosis. By a punch biopsy of the iliac crest a low-grade non-Hodgkin's lymphoma could be identified as the cause for this generalized amyloidosis.


CONCLUSIONS
In the present case, the reason for these unusual hepatorenal symptoms with unclear cholestasis over years as the first clinical symptom and a succeeding progressive renal insufficiency with proteinuria could be found by the use of kidney biopsy and extending the analysis of a liver sample taken by biopsy 1 year ago. Immunoglobulin light chains produced by a low-grade non-Hodgkin's lymphoma caused a generalized amyloidosis type A lambda.


CONCLUSIONS
As a consequence, by an occurrence of unusual hepatorenal symptoms with cholestasis and progressive renal failure, amyloidosis should be considered as a pathogenetic factor.
Medizinische Klinik IV, Universität Erlangen-Nümberg.
H
H
Frank
M
M
Krammer
W
W
Fierlbeck
R
R
Riess
H
H
Geiger
D000368:Aged; D000686:Amyloidosis / Q000175:diagnosis* / Q000473:pathology; D001706:Biopsy; D001853:Bone Marrow / Q000473:pathology; D003937:Diagnosis, Differential; D006530:Hepatorenal Syndrome / Q000175:diagnosis / Q000209:etiology* / Q000473:pathology; D006801:Humans; D007668:Kidney / Q000473:pathology; D007674:Kidney Diseases / Q000175:diagnosis* / Q000473:pathology; D008099:Liver / Q000473:pathology; D008107:Liver Diseases / Q000175:diagnosis* / Q000473:pathology; D016393:Lymphoma, B-Cell / Q000175:diagnosis* / Q000473:pathology; D008228:Lymphoma, Non-Hodgkin / Q000175:diagnosis* / Q000473:pathology; D008297:Male
Ungewöhnliche Ursache einer hepatorenalen Symptomatik.