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Liver and intrahepatic bile ducts-nontumor

Metabolic diseases

Familial apolipoprotein A-I amyloidosis


Reviewers: Komal Arora, M.D. (see Reviewers page)
Revised: 24 April 2012, last major update April 2012
Copyright: (c) 2004-2012, PathologyOutlines.com, Inc.

General
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● Apo A-I is major protein constituent of plasma high density lipoprotein; is synthesized in liver and intestine
● At least 10 different amyloidogenic Apo A-I mutations exist

Treatment
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● Liver transplant slows disease progression
● Case reports of 2 sisters with liver transplants (Mod Pathol 2001;14:577)

Micro description
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● Often striking liver parenchymal involvement by large patches of amyloid replacing hepatic lobules, separating hepatic cords, accentuation around central veins, involving portal triads, hilar soft tissue
● No involvement of hilar nerve branches or vagus nerve

Micro images
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Extensive amyloid deposition


Congo red stain

       
Various images

Positive stains
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● Congo red

Additional references
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Amyloid 2003;10:215, J Mol Diagn 2009;11:257

End of Liver and intrahepatic bile ducts-nontumor > Metabolic diseases > Familial apolipoprotein A-I amyloidosis


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