PRIMARY AMYLOIDOSIS AND SEVERE INTRAHEPATIC CHOLESTATIC JAUNDICE

Liver involvement in systemic amyloidosis is frequent but is rarely of clinical importance. Five patients with severe cholestatic jaundice a re described and an additional 20 from published reports are reviewed. The most frequent presenting symptoms were lethargy and abdominal pai n, which were present for a median of 11 months before the onset of ja undice. Hepatomegaly, usually marked, was present in 92%, with ascites in 56% of the cases. The serum bilirubin concentration was noticeably high and the serum globulin low. Histology of the Liver showed consid erable perisinusoidal deposition with a slight predilection for the pe riportal area. Two patients presented with predominant centrilobular d eposition. Congo red staining was not uniformly positive. A variety of treatment regimens was tried but median survival was only three month s from the onset of jaundice.