Primary liver cancer in two sisters in Holland with intermittent acute porphyria

S Afr Med J. 1997 Jun;87(6):731-2.


Two sisters living in Holland, with a niece now living in South Africa, were reported in 1958 to have inherited intermittent acute porphyria (IAP). In 1994 both sisters died from primary liver cancer. Other reports have also noted an increased mortality from carcinoma of the liver in porphyrics. Porphyria variegata has a high prevalence in white and coloured South Africans, and it would be relatively easy to ascertain whether those who have inherited the gene for this disorder, in South Africa, have a higher than reported mortality from liver cancer. If they do, consideration should be given to ways to reduce their risk of developing and dying from this cancer.

MeSH terms

  • Barbiturates / adverse effects
  • Contraindications
  • Female
  • Humans
  • Liver Neoplasms / complications*
  • Male
  • Netherlands
  • Pedigree
  • Porphyria, Acute Intermittent / complications*
  • Porphyria, Acute Intermittent / genetics
  • Risk Factors


  • Barbiturates