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Erythropoiesis and iron metabolism in dominant erythropoietic protoporphyria

S. Alexander Holme, Mark Worwood, Alexander V. Anstey, George H. Elder and Michael N. Badminton

Data supplements

  • Supplemental materials for: Holme et al

    Files in this Data Supplement:

    • Table S1. Red cell indices in 178 patients with dominant EPP (PDF, 17.8 KB) -
      Figures are mean ± SD for 98 females aged 6 � 87 years, median 35 years; 80 males aged 8 � 87 years, median 36 years. Anemia was defined as Hb less than 12.0 g/dL (females) or less than 13.0 g/dL (males) for adults and less than the lower limit of the age-related Hb range for children (Hinchcliffe RF. Reference values. In: Pediatric Hematology. Lilleyman JS, Hann IM, Blanchette VS, eds. London, Churchill Livingstone; 1999: 1-20.). For comparison of anaemia vs. no anaemia groups: **P < 0.01; ***P < 0.001. Twenty four of our female patients aged over 16 years had been prescribed oral iron preparations though only 5 patients were taking them at the time of investigation. The mean Hb in these patients was 11.3 ± 1.0 g/dL compared with 12.2 ± 0.8 g/dL for those who had never taken iron preparations (P < 0.01). There was no correlation between Hb and age in adults and the percentages of anemic patients were 43% for those aged under 19 years and 41% for those over 18 years.

    • Table S2. Iron status of 178 patients with dominant EPP (PDF, 58.2 KB) -
      Figures are medians and ranges or, for TIBC, means ± SD. Iron insufficent was defined as TS < 16% and/or sFn < 15 µg/L. For comparisons of percentages of male and female patients: *P < 0.05; **P < 0.001. In both sexes, sFn correlated with Hb (females: rs = 0.459, P < 0.001; males: rs = 0.415, P < 0.001). TS correlated with Hb in females (rs = 0.418, P < 0.001) but not in males. In neither sex was there any significant correlation between Hb and TIBC. Erythrocyte protoporphyrin concentrations showed no correlation with sFn, TIBC or TS in either sex.

Article Figures & Data

Figures

  • Figure 1

    Hemoglobin, serum ferritin, and serum soluble transferrin receptor-1 concentrations in male patients with dominant EPP. (A). Hemoglobin concentrations in 66 male patients with dEPP aged 16 years or older (■) and in a sample of 5206 men aged 16 years or older from the English population17 (□). (B) Serum ferritin concentrations in 66 male patients with dEPP aged 16 years or older (■) and in 612 male first-time blood donors from south Wales aged 17 to 62 years16 (□). (C) Serum soluble transferrin receptor-1 concentrations in 61 male patients with dEPP aged 16 years or older (18.6 ± 5.1 nM) (■) and in 225 hematologically healthy male and female subjects from the United States aged 17 to 97 years18 (□) assayed using the same method.

Tables

  • Table 1

    Comparison of indicators of iron status in male patients with dEPP and male first-time blood donors

    Serum iron indicesEPP patients, n = 67First-time blood donors, n = 611
    sFe, μM15.1 ± 6.6*16.7 ± 6.0
    TIBC, μM63.0 ± 6.954.5 ± 10.0
    TS, %23.9 ± 10.331.1 ± 10.9
    sFn, μg/L37 (10-119)101 (35-220)
    • Values are means plus or minus SD, except for sFn, which are medians and 95% ranges. EPP patients are males aged 16 to 77 years who have never been prescribed iron supplements. Blood donors are male first-time donors aged 17 to 62 years from South Wales15; samples for analysis were obtained prior to first donation. Only TIBC showed any correlation with sFn (rs = −0.412, P < .001).

    • * P value is not significant.

    • P < .001 compared with donors.