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Iron overload secondary to cirrhosis: a mimic of hereditary haemochromatosis?
Murad Abu Rajab1, Leana Guerin, Pauline Lee
1Division of Gastroenterology-Hepatology, University of Iowa Roy J. and Lucille A. Carver College of Medicine, Iowa City, IA, USA.
Insights
Cirrhosis can cause hepatic iron overload, potentially leading to iron buildup in the pancreas and heart. This extrahepatic iron deposition in cirrhosis patients mimics hemochromatosis and may cause complications.
Area of Science:
- Hepatology
- Gastroenterology
- Pathology
Background:
- Hepatic iron deposition is common in cirrhosis, often unrelated to hereditary hemochromatosis.
- The extent of extrahepatic iron deposition in cirrhosis is not well-characterized.
Purpose of the Study:
- To investigate the association between hepatic iron deposition and extrahepatic organ iron deposition in cirrhosis patients.
- To determine if cirrhosis-related hepatic haemosiderosis correlates with iron accumulation in organs beyond the liver.
Main Methods:
- Autopsy records of 104 adult cirrhosis patients were reviewed.
- Histochemical staining assessed storage iron in the liver, heart, pancreas, and spleen.
- HFE genotyping was performed on individuals with significant iron deposition.
Main Results:
- 46% of patients had stainable hepatic iron; most cases were mild (2+ or less).
- Six patients (approx. 6%) exhibited heavy hepatic iron deposition (4+).
- These six patients also had pancreatic iron, and five had cardiac iron, without HFE gene mutations.
Conclusions:
- Heavy hepatic iron deposition in cirrhosis is frequently linked to pancreatic and cardiac iron.
- This iron deposition pattern resembles hemochromatosis, despite a lack of HFE mutations.
- Cirrhosis patients with significant hepatic haemosiderosis may face risks of extrahepatic iron overload complications.
Aims:
Hepatic iron deposition unrelated to hereditary haemochromatosis is common in cirrhosis. The aim of this study was to determine whether hepatic haemosiderosis secondary to cirrhosis is associated with iron deposition in extrahepatic organs.
Methods And Results:
Records of consecutive adult patients with cirrhosis who underwent autopsy were reviewed. Storage iron was assessed by histochemical staining of sections of liver, heart, pancreas and spleen. HFE genotyping was performed on subjects with significant liver, cardiac and/or pancreatic iron. The 104 individuals were predominantly male (63%), with a mean age of 55 years. About half (46%) had stainable hepatocyte iron, 2+ or less in most cases. In six subjects, there was heavy iron deposition (4+) in hepatocytes and biliary epithelium. All six of these cases had pancreatic iron and five also had cardiac iron. None of these subjects had an explanatory HFE genotype.
Conclusions:
In this series, heavy hepatocyte iron deposition secondary to cirrhosis was commonly associated with pancreatic and cardiac iron. Although this phenomenon appears to be relatively uncommon, the resulting pattern of iron deposition is similar to haemochromatosis. Patients with marked hepatic haemosiderosis secondary to cirrhosis may be at risk of developing extrahepatic complications of iron overload.
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