Cancer risk in patients with hereditary hemochromatosis and in their first-degree relatives
(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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https://lup.lub.lu.se/record/899626
- author
- organization
- publishing date
- 2003
- type
- Contribution to journal
- publication status
- published
- subject
- in
- Gastroenterology
- volume
- 125
- issue
- 6
- pages
- 1733 - 1741
- publisher
- Elsevier
- external identifiers
-
- wos:000187177600025
- pmid:14724826
- scopus:10744232491
- ISSN
- 1528-0012
- DOI
- 10.1053/j.gastro.2003.09.035
- language
- English
- LU publication?
- yes
- id
- ecf8e70a-54a9-4e7c-99f4-64fe9534345d (old id 899626)
- date added to LUP
- 2016-04-01 12:38:43
- date last changed
- 2022-03-29 03:41:27
@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 = {{Elsevier}}, 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}}, doi = {{10.1053/j.gastro.2003.09.035}}, volume = {{125}}, year = {{2003}}, }