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Liver and intrahepatic bile ducts-nontumor

Metabolic diseases

Erythropoietic protoporphyria


Reviewers: Komal Arora, M.D. (see Reviewers page)
Revised: 23 April 2012, last major update April 2012
Copyright: (c) 2004-2012, PathologyOutlines.com, Inc.

General
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● Excess production of free protoporphyrin from the bone marrow, most commonly due to deficiency of the enzyme ferrochelatase; causes characteristic cutaneous photosensitivity, and in a minority of patients leads to end-stage liver disease necessitating liver transplantation (J Inherit Metab Dis 2011;34:539, World J Gastroenterol 2010;16:4526)
● Paracrystalin protoporphyrin deposition in hepatocytes and precipitation in the biliary canaliculi
● Prognosis depends on extent of liver disease (Orphanet J Rare Dis 2009 Sep 10;4:19)

Diagrams
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Heme biosynthetic pathway

Gross images
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Explanted liver

Micro description
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● Deep brown colored bile in canaliculi, bile ducts and Kupffer cells with red “Maltese cross” under polarized light

Micro images
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Birefringence of pigment deposits due to protoporphyrin crystals


Diffuse dark discoloration and micronodular cirrhosis

Electron microscopy description
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● Star-burst crystalline array

End of Liver and intrahepatic bile ducts-nontumor > Metabolic diseases > Erythropoietic protoporphyria


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