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Cancer risk in patients with hereditary hemochromatosis and in their first-degree relatives

Elmberg, M; Hultcrantz, R; Ekbom, A; Brandt, L; Olsson, S; Olsson, R; Lindgren, Stefan LU ; Loof, L; Stal, P and Wallerstedt, S, et al. (2003) In Gastroenterology 125(6). p.1733-1741
Abstract
Background & Aims: Iron overload may be carcinogenic. Patients with hereditary hemochromatosis (HH) are reportedly at a 20-200-fold risk of intrahepatic cancer, but the reported risks for nonhepatobiliary cancers are conflicting. The risk of cancer in heterozygous individuals (estimated allele frequency, 1/10 to 1/20) is unknown. This study aimed to better assess these risks. Methods: We performed a population-based cohort study of 1847 Swedish patients with HH and 5973 of their first-degree relatives using nationwide, population-based health and census registers. We used standardized incidence ratios (SIRs) as relative risk. Results: With 62 liver cancers and 128 nonhepatobiliary cancers, patients with HH were at a 20-fold risk of... (More)
Background & Aims: Iron overload may be carcinogenic. Patients with hereditary hemochromatosis (HH) are reportedly at a 20-200-fold risk of intrahepatic cancer, but the reported risks for nonhepatobiliary cancers are conflicting. The risk of cancer in heterozygous individuals (estimated allele frequency, 1/10 to 1/20) is unknown. This study aimed to better assess these risks. Methods: We performed a population-based cohort study of 1847 Swedish patients with HH and 5973 of their first-degree relatives using nationwide, population-based health and census registers. We used standardized incidence ratios (SIRs) as relative risk. Results: With 62 liver cancers and 128 nonhepatobiliary cancers, patients with HH were at a 20-fold risk of liver cancer (SIR, 21; 95% confidence interval [Cl], 16-22) but an almost unaltered risk of all other cancers (SIR, 1.2; 95% Cl, 1.0-1.4), including nonelevated risks for several gastrointestinal tract cancers. At 10 years of follow-up, the absolute risk of liver cancer was 6% among men and 1.5% among women. With 21 liver cancers and 508 nonhepatobiliary cancers, first-degree relatives were at an unaltered risk of extrahepatic cancer (SIR, 1.0; 95% Cl, 0.9-1.1, including unelevated risks for gastrointestinal cancers) but at a modest and historic increased risk of hepatobiliary cancer (SIR, 1.5; 95% Cl, 1.0-2.4), the histopathologic spectrum of which differed from the patients. Conclusions: Patients (particularly men) with HH are at increased risk for hepatocellular cancer, although the magnitude of the risk is lower than previous estimates. Overall cancer risk in first-degree relatives does not seem to be increased. (Less)
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Contribution to journal
publication status
published
subject
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Gastroenterology
volume
125
issue
6
pages
1733 - 1741
publisher
W B Saunders
external identifiers
  • wos:000187177600025
  • pmid:14724826
  • scopus:10744232491
ISSN
1528-0012
DOI
10.1053/j.gastro.2003.09.035
language
English
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yes
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ecf8e70a-54a9-4e7c-99f4-64fe9534345d (old id 899626)
date added to LUP
2008-01-10 19:40:06
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2018-10-03 11:38:41
@article{ecf8e70a-54a9-4e7c-99f4-64fe9534345d,
  abstract     = {Background & Aims: Iron overload may be carcinogenic. Patients with hereditary hemochromatosis (HH) are reportedly at a 20-200-fold risk of intrahepatic cancer, but the reported risks for nonhepatobiliary cancers are conflicting. The risk of cancer in heterozygous individuals (estimated allele frequency, 1/10 to 1/20) is unknown. This study aimed to better assess these risks. Methods: We performed a population-based cohort study of 1847 Swedish patients with HH and 5973 of their first-degree relatives using nationwide, population-based health and census registers. We used standardized incidence ratios (SIRs) as relative risk. Results: With 62 liver cancers and 128 nonhepatobiliary cancers, patients with HH were at a 20-fold risk of liver cancer (SIR, 21; 95% confidence interval [Cl], 16-22) but an almost unaltered risk of all other cancers (SIR, 1.2; 95% Cl, 1.0-1.4), including nonelevated risks for several gastrointestinal tract cancers. At 10 years of follow-up, the absolute risk of liver cancer was 6% among men and 1.5% among women. With 21 liver cancers and 508 nonhepatobiliary cancers, first-degree relatives were at an unaltered risk of extrahepatic cancer (SIR, 1.0; 95% Cl, 0.9-1.1, including unelevated risks for gastrointestinal cancers) but at a modest and historic increased risk of hepatobiliary cancer (SIR, 1.5; 95% Cl, 1.0-2.4), the histopathologic spectrum of which differed from the patients. Conclusions: Patients (particularly men) with HH are at increased risk for hepatocellular cancer, although the magnitude of the risk is lower than previous estimates. Overall cancer risk in first-degree relatives does not seem to be increased.},
  author       = {Elmberg, M and Hultcrantz, R and Ekbom, A and Brandt, L and Olsson, S and Olsson, R and Lindgren, Stefan and Loof, L and Stal, P and Wallerstedt, S and Almer, S and Sandberg-Gertzen, H and Askling, J},
  issn         = {1528-0012},
  language     = {eng},
  number       = {6},
  pages        = {1733--1741},
  publisher    = {W B Saunders},
  series       = {Gastroenterology},
  title        = {Cancer risk in patients with hereditary hemochromatosis and in their first-degree relatives},
  url          = {http://dx.doi.org/10.1053/j.gastro.2003.09.035},
  volume       = {125},
  year         = {2003},
}