title: Liver pathology in genetic hemochromatosis: a review of 135 homozygous cases and their bioclinical correlations.
abstract: Liver pathology was assessed in 135 patients with well-defined genetic hemochromatosis ranging from mild disease to severe overload. Three lesions were clearly linked to iron-overload intensity--scarce sidero-necrosis, mild inflammation, and progressive fibrosis. Iron-free foci made of typical or dysplastic hepatocytes were found in 7.4% of the cases. An original grading allowed a reliable quantification of iron and the study of cellular and lobular distribution of iron, which permitted (a) the accurate identification of a decreasing iron gradient in hepatocytes from zone 1 to zone 3 in all cases, (b) the definition of a threshold hepatocytic/mesenchymal iron ratio related to the appearance of sidero-necrosis and to the development of fibrosis, and (c) demonstration that non-iron-related factors (mainly alcoholism) could shift iron from hepatocytes to sinusoidal cells without an increase in the total liver iron amount. This study provides a dynamic view of the iron overload process and suggests that sidero-necrosis and progressive sinusoidal iron overload play a role in the development of fibrosis in human genetic hemochromatosis.
authors.affiliation: Clinique des Maladies du Foie, Inserm U49, Rennes, France.
authors.forename: Y M
authors.initials: YM
authors.lastname: Deugnier
authors.forename: O
authors.initials: O
authors.lastname: Loréal
authors.forename: B
authors.initials: B
authors.lastname: Turlin
authors.forename: D
authors.initials: D
authors.lastname: Guyader
authors.forename: H
authors.initials: H
authors.lastname: Jouanolle
authors.forename: R
authors.initials: R
authors.lastname: Moirand
authors.forename: C
authors.initials: C
authors.lastname: Jacquelinet
authors.forename: P
authors.initials: P
authors.lastname: Brissot
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D005260:Female; D006432:Hemochromatosis / Q000235:genetics / Q000473:pathology*; D006720:Homozygote; D006801:Humans; D007501:Iron / Q000032:analysis; D008099:Liver / Q000737:chemistry / Q000473:pathology*; D008297:Male; D008875:Middle Aged
chemical_list: D007501:Iron