Acute Hepatic Porphyrias and Primary Liver Cancer

Abstract

Acute hepatic porphyrias (acute intermittent porphyria, variegate porphyria, and hereditary coproporphyria) are inherited disorders caused by partial enzyme defects in heme biosynthesis.1 Two retrospective studies and one case–control study have suggested an association between primary liver cancer and acute hepatic porphyrias.2-4 We present data from a prospective cohort study that assessed the crude annual incidence rate of primary liver cancer in 650 French carriers of the gene for acute hepatic porphyria followed for seven years. We compared this incidence with the incidence of primary liver cancer in France using the standardized rate ratio after indirect standardization. National age- and sex-specific incidences of primary liver cancer were provided by the French network of cancer registries.5 Of the patients with acute hepatic porphyria, 71 were lost to follow-up and 18 died. None of the deaths were ascribed to primary liver cancer.