Table I.
Median measurements of iron status and erythropoiesis in patients with thalassaemia major (TM), sickle cell disease (SCD), Diamond-Blackfan anaemia (DBA): differences between medians (±first and third quartile values) were tested using non-parametric analysis of variance (ANOVA; Kruskal-Wallis) with a Dunn’s post-test.
| Pre-transfusion Values Median ± 1st & 3rd quartile values |
TM (n = 5) | SCD (n = 5) | DBA (n = 5) | Control (n = 5) | ANOVA P |
|---|---|---|---|---|---|
| NTBI (μmol/l) | 1·68 (1·21, 1·79)a | −0·23 (−1·83, 0·71)b | 2·50 (1·96, 3·17)a | −2·63 (−2·71, −2·63)c | 0·001 |
| Transferrin saturation (%) | 100 (100, 100)a | 49·7 (38·2, 91·4)b | 100 (94·7, 100)a | 36·0 (29·4, 39·6)c | 0·002 |
| LPI (μmol/l) | 1·30 (0·86, 2·10)a | 0·05 (−0·05, 0·79)a,b | 0·86 (0·33, 1·67)a,b | 0·01 (−0·05, 0·10)b | 0·037 |
| LIC (R2*) (mg/g dry weight) | 18·3 (8·3)a | 26·0 (8·0)a | 6·1 (24·2)a | 1·7* | 0·5 |
| Transferrin (g/l) | 1·94 (1·69, 1·94)a | 2·44 (2·10, 3·06)a | 2·67 (2·32, 3·22)a | 4·45 (3·99, 5·8)b | 0·008 |
| Ferritin (μg/l) | 3251 (4680)a,b | 12000 (27168)a | 2150 (13984)a,b | 32 (109)b | 0·003 |
| sTfR (nmol/l)† | 14·9 (6·30, 19·7)a | 8·4 (7·70, 14·3)a,b | 0·00 (0·0, 0·55)* c | 3·2 (2·75, 3·70)b,c | 0·001 |
| GDF15 (pg/ml) | 5504 (2965, 11067)a | 634 (527, 3690)a,b | 467 (332, 3078)b | 279 (272, 307)b | 0·012 |
| Erythropoietin (miu/ml) | 41·0 (11·0, 108·0)b | 28·0 (21·0, 38·0)b | 2004 (1162, 3474)a | 7·0 (5·0, 8·5)b | 0·002 |
| Hepcidin (nmol/l) | 3·97 (21·2)b | 24·3 (28·5)a | 28·7 (36·3)a | 0·81 (2·57)b | 0·002 |
| SLC40A1/RPL27 (mRNA)‡ ×1000) | 41 (32·2, 195)a | 25 (5, 848)a | 75 (18, 165)a | 56 (21, 476)a | 0·820 |
| Hepcidin/Ferritin‡ | 1·7 (0·71, 5·57)a | 1·6 (1·06, 2·9)a | 12·4 (3·2, 66·4)b | 23·2 (15·2, 93·0)b | 0·023 |
| hsCRP (mg/l) | 0·9 (0·25, 3·35)a,b | 3·2 (2·31, 5·25)a | 1·8 (0·3, 9·05)a,b | 0·34 (0·21, 0·75)b | 0·041 |
NTBI, non-transferrin bound iron; LPI, labile plasma iron; LIC, liver iron concentration; sTfR, soluble transferrin receptor; GDF15, growth differentiation factor 15; hsCRP, high-sensitivity C-reactive protein.
The control LIC is a calculated value, determined from Wood et al (2005).
In 3 out of 5 DBA patients, sTfR levels were below detection limit (<0·5 nmol/l).
Monocyte SLC40A1/RPL27 mRNA and Hepcidin/Ferritin are reported at ×1000.
Within a row, groups not sharing the same letter are significantly different from each other (5% procedure wise error rate).