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Blood, Vol. 110, Issue 12, 4108-4110, December 1, 2007
Erythropoiesis and iron metabolism in dominant erythropoietic protoporphyria
Blood Holme et al.
110: 4108
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.
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