Liver pathology in genetic hemochromatosis: A review of 135 homozygous cases and their bioclinical correlations

Deugnier, Yves; Loréal, Olivier; Turlin, Bruno; Guyader, Dominique; Jouanolle, H.; Moirand, Romain; Jacquelinet, Christian; Brissot, Pierre

doi:10.1016/0016-5085(92)90331-r

Cited by 286 publications

(199 citation statements)

References 24 publications

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“…In these groups, the necroinflammatory activity (hepatocyte necrosis and ballooning, lobular and portal inflammatory infiltration), the presence of Mallory bodies, or iron deposits were assessed. 14 The tumor characteristics (including differentiation) were also determined for all patients. 15 Data are expressed as mean Ϯ standard deviation (SD).…”

Section: Methodsmentioning

confidence: 99%

Obesity and diabetes as a risk factor for hepatocellular carcinoma

et al. 2004

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Ten percent of patients who undergo resection for hepatocellular carcinoma (HCC) associated with chronic liver disease have no detectable cause for this underlying liver disease. Recent studies have shown that patients with cryptogenic chronic liver disease frequently have risk factors for nonalcoholic fatty liver disease (NAFLD). This study examines the incidence of risk factors for NAFLD in patients with chronic liver disease who underwent resection for HCC. Among 210 patients with chronic liver disease who underwent resection for HCC, 18 (8.6%) had no identifiable cause for the underlying liver disease. These patients were assessed for obesity, diabetes mellitus, and histological features of the tumor and the adjacent liver parenchyma. Comparisons were made with matched patients with alcohol-and chronic-viral-hepatitis-related HCC. The prevalence of obesity (50% vs. 17% vs. 14%), diabetes (56% vs. 17% vs. 11%), aspartate aminotransferase / alanine aminotransferase ratio < 1 (50% vs. 19% vs. 17%), and steatosis > 20% (61% vs. 17% vs. 19%) was significantly higher in patients with cryptogenic liver disease than in patients with alcohol abuse and chronic viral hepatitis (P < 0.0001 for each). Well-differentiated tumors were significantly more common in patients with cryptogenic liver disease (89% vs. 64% in patients with alcohol-related HCC vs. 55% in patients with chronic viral hepatitis-related HCC, P < 0.0001). In conclusion, the hypothesis that obesity and diabetes mellitus may be important risk factors for cryptogenic chronic liver disease in patients with HCC is supported by the analysis of surgically treated patients. Whether HCC is primarily related to obesity and diabetes mellitus or secondarily to a NAFLD-like parenchymal lesions remains to be clarified. (Liver Transpl 2004;10: S69 -S73.)

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Section: Methodsmentioning

confidence: 99%

Obesity and diabetes as a risk factor for hepatocellular carcinoma

et al. 2004

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“…25,26 Hemosiderin is insoluble and particulate in nature and will appear granular with iron stains. 26 Alternatively, iron may be deposited in the form of ferritin which is soluble and characterized by diffuse, non-granular light blue staining of the hepatocyte or macrophage cytoplasm and is very non-specific and not characteristic of genetic hemochromatosis.…”

Section: Histopathology Of Adult Hereditary Hemochromatosismentioning

confidence: 99%

“…Macrophage iron is linked to progressive fibrosis. 26 Precirrhotic portal and periportal fibrosis takes on a 'holly leaf' configuration. When cirrhosis is present, it is bland in nature with fine fibrous tissue septa surrounding regenerative nodules.…”

Section: Histopathology Of Adult Hereditary Hemochromatosismentioning

confidence: 99%

Iron overload syndromes and the liver

Batts

2007

Modern Pathology

132

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“…Excess alcohol consumption can, however, affect the distribution of iron stores within the liver, which may cause diagnostic confusion, but does not affect the total amount of iron stores. 52 Measurement of the urinary excretion of iron following an infusion of desferrioxamine has in the past been used to diagnose haemochromatosis. However, this test is much less reliable than serum ferritin, TS and hepatic iron concentration in diagnosing haemochromatosis and is no longer in routine use.…”

Section: Diagnosismentioning

confidence: 99%

the screening, diagnosis and optimal management of haemochromatosis

George

Powell

1997

Aliment Pharmacol Ther

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Haemochromatosis was first recognized as a disease entity over a century ago and its hereditary nature recognized over 60 years ago. However it was only in late 1996 that the haemochromatosis gene was cloned and a single C282Y mutation confirmed as being the cause of all HLA‐linked iron overload in Caucasian populations. Haemochromatosis is common, occurring in approximately 1 in 300 people in Caucasian populations, and untreated can cause serious morbidity and early death. However, the disease remains much underdiagnosed for reasons such as lack of awareness of the disease, the presence of normal liver function tests and the lack or non‐specific nature of symptoms. A␣commercially available DNA‐based test for the haemochromatosis gene is likely to be available in the near future but its place in the diagnosis and management of the disorder is not yet clear. Assessment of body iron stores by measurement of serum ferritin and transferrin saturation, hepatic iron stores and hepatic architecture by liver biopsy will remain important in the future. The haemochromatosis mutation itself has as yet no known influence on morbidity other than via iron loading and organ failure, in particular, hepatic cirrhosis. Thus, diagnosing patients before the development of hepatic cirrhosis is crucial because iron depletion by venesection treatment before the development of cirrhosis results in a normal life expectancy.

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Liver pathology in genetic hemochromatosis: A review of 135 homozygous cases and their bioclinical correlations

Cited by 286 publications

References 24 publications

Obesity and diabetes as a risk factor for hepatocellular carcinoma

Obesity and diabetes as a risk factor for hepatocellular carcinoma

Iron overload syndromes and the liver

the screening, diagnosis and optimal management of haemochromatosis

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