Unusual hepatorenal symptoms in amyloidosis

Case Report: A 65-year-old patient with normal blood pressure had an exclus ive elevation of the cholestasis enzymes (alkaline phosphatase 297 U/1, gam ma-GT 315 U/1) 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 h epatitis"; DD drug-induced or toxic lesions. Serological tests gave no supp ort for an acute hepatitis. Intra- or extrahepatic cholestasis could not be proved neither by ultrasound nor by an endoscopic retrograde cholangiopanc reatography. Since November 1995 serum creatinine increased gp to 17 mg/dl (March 1995 1.1 mg/dl) and proteinuria (2.1 g/d) developed. Due to worsenin g of renal function (serum creatinine 2.8 mg/dl) and increasing proteinuria (3.5 g/d) without nephrotic syndrome, a kidney biopsy was performed. Histo logically an amyloidosis (type A lambda) was proven, involving glomerula, k idney vessels and tubules. Further biopsies from the stomach and the duoden um showed profound infiltration of the mucosa and submucosa with amyloid. T herefore, 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. Discussion: In the present case, the reason for these unusual hepatorenal s ymptoms with unclear cholestasis over years as the first clinical symptom a nd a succeeding progressive renal insufficiency with proteinuria could be f ound by the use of kidney biopsy and extending the analysis of a liver samp le taken by biopsy 1 year ago. Immunoglobulin light chains produced by a lo w-grade non-Hodgkin's lymphoma caused a generalized amyloidosis type A lamb da. Conclusion: As a consequence, by an occurrence of unusual: hepatorenal symp toms with cholestasis and progressive renal failure, amyloidosis should be considered as a pathogenetic factor.