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Although hepatic involvement is common in all forms of amyloidosis, jaundice is infrequent and usually mild. We report a case of a 74-yr-old man with severe intrahepatic cholestasis and hepatic amyloid. The topography of amyloid deposition was unusual, in that deposits were confined predominantly to the portal tract. They appeared as a dense eosinophilic infiltrate that stained with congo red. A review of the English-language literature revealed only 18 prior patients with hepatic amyloid associated with severe intrahepatic cholestasis. The histology of only one of these patients showed a similar distribution of amyloid deposits.
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