Prevalence of abnormal iron studies in heterozygotes for hereditary hemochromatosis: an analysis of 255 heterozygotes

Am J Hematol. 1994 Feb;45(2):146-9. doi: 10.1002/ajh.2830450210.

Abstract

Iron studies were compared in 434 patients from 80 hemochromatosis families classified as putative homozygotes, heterozygotes, and normal by HLA typing. There were 28 of 255 (11%) heterozygotes with an elevated serum ferritin and 22 of 255 (8.6%) with an elevated transferrin saturation. Serum ferritin (140 +/- 10.2 micrograms/liter; mean +/- standard error) was greater in heterozygotes than in normal subjects (87 +/- 8.5 micrograms/liter; P < .05, Mann Whitney test). Transferrin saturation was greater in heterozygotes (38% +/- 0.88%) than in normal patients (29% +/- 1.1%; P < .0001). Mean hepatic iron concentration was 54 +/- 6 mumol/g (n = 17), and the hepatic iron index was < 2 in these patients. Most heterozygotes for hemochromatosis have a normal serum ferritin and transferrin saturation. Heterozygotes with minor elevations in serum ferritin or transferrin saturation do not have significant iron overload as assessed by hepatic iron concentration.

MeSH terms

  • Female
  • Ferritins / blood
  • HLA-A Antigens / analysis
  • HLA-B Antigens / analysis
  • Hemochromatosis / blood*
  • Hemochromatosis / genetics*
  • Heterozygote*
  • Histocompatibility Testing
  • Humans
  • Iron / blood*
  • Liver / chemistry
  • Male
  • Middle Aged
  • Transferrin / metabolism

Substances

  • HLA-A Antigens
  • HLA-B Antigens
  • Transferrin
  • Ferritins
  • Iron