Primary amyloidosis andsevereintrahepatic cholestatic jaundice

Liverinvolvement insystemic amyloidosisisfrequent butisrarely ofclinical importance. Fivepatients withsevere cholestatic jaundice aredescribed andan additional 20frompublished reports are reviewed. Themostfrequent presenting symptomswerelethargy andabdominal pain,whichwerepresentfora median of11monthsbefore theonsetofjaundice. Hepatomegaly, usually marked, waspresentin92%,withascites in56% ofthe cases. Theserumbilirubin concentration was noticeably highand the serum globulin low.Histology oftheliver showed considerable perisinusoidal deposition witha slight predilection fortheperiportal area.Two patients presented with predominantcentrilobular deposition. Congoredstaining was notuniformly positive. A variety oftreatmentregimens wastried butmediansurvival was only threemonthsfromtheonsetofjaundice. (Gut1994; 35:1322-1325)