Acase report of primary amyloidosis with prominent hepatic involvement

primary amyloidosis is a rare disrder wihich is diagnosed by extracellular deposition of proteinaceous material in different organs.in this report,a case of this disease with prominent hepatic involvement is presented.the case is a ۶۳ years old male refered with abdominal enlargement,weight loss,generalized pruritus,anorexia and vague abdominal pain started from six months ago.on physical examination excoriation mark and hepatomegaly with liver span about ۲۰cm were noted.jaundice,splenomegaly,ascitis,edema and skin lesions were absent.based on labaratory report there were an increase in alkaline phosphatase ۳ times above the norman range and mild elevation in aminotranferases.proteinuria was ۲.۷ grams in ۲۴hr uring collection.liver biopsy revealed homogenous and amorphous deposition of extracellular material with green birefregence in congo red staining.therefore the diagnosis of primary amyloidosis after exclusion of other causes was comfirmed.

کلیدواژه ها:

primary amyloidosis