Demographic, iron, erythropoietic, and inflammatory indices for patients with HbE β-thalassemia overall and stratified by severe and moderate phenotype
| Parameter . | All patients (n = 69; 42 female) . | Severe (n = 28; 17 female) . | Moderate (n = 41; 25 female) . | P for difference between moderate/severe . | Local controls (n = 25) . |
|---|---|---|---|---|---|
| Age, years* | 26.6 (3-61) | 17.5 (3-28) | 32.8 (9-61) | <.0001† | 16.6 (16.0, 17.2) |
| Hb, g/dL* | 6.2 (4.4-8.3) | 5.8 (4.4-7.6) | 6.4 (4.9-8.3) | .1397 | 15.0 (14.5, 15.5)‡ |
| HbF, %* | 26.0 (6.2-51.2) | 24.1 (6.2-42.4) | 27.3 (6.4-51.2) | .3226 | 0.27 (0.19, 0.39)‡ |
| Total transfusions* | 47.6 (0, 143) | 59.3 (3, 143) | 39.7 (0, 123) | .0377† | |
| Ferritin, mg/L** | 947 (143, 9790) | 1356 (328, 9790) | 732 (143, 3260) | .0021† | 48.2 (38, 61.2)‡ |
| Liver iron, mg/gdwt** | 7.21 (0.7, 54.2) | 11.3 (1, 54.2) | 5.30 (0.7, 29.0) | .0006† | |
| NTBI, µM/L* | 4.68 (−2.67, 23.75) | 5.16 (−1.67, 10.27) | 4.36 (−2.67, 23.75) | .4456 | |
| Hepcidin, ng/mL** | 2.06 (0.1, 51.8) | 2.24 (0.1, 51.8) | 1.95 (0.1, 36.3) | .7441 | 28.23 (18.83, 42.3)‡ |
| sTfR, nM/L** | 7.83 (1, 35.3) | 6.51 (1, 35.3) | 8.87 (1.4, 35.3) | .1472 | |
| EPO, mg/L** | 158 (28.7, 2196.7) | 159 (28.7, 2196.7) | 158 (34.5, 1296.5) | .9915 | |
| CRP, mg/L** | 1.61 (0.20, 34.23) | 1.66 (0.23, 34.23) | 1.58 (0.20, 13.01) | .8433 | |
| GDF-15, pg/mL,** | 8407 (1920, 60 229) | 7011 (2125, 19 031) | 9517 (1920, 60 229) | .0549 | |
| Labile plasma iron, µM/L* | 4.01 (−1.07, 13.61) | 3.60 (0.1, 7.7) | 4.29 (−1.07, 13.61) | .9320 | |
| Transferrin SAT, %* | 90.9 (31.9, 100.0) | 97.3 (48.3, 100.0) | 86.6 (31.9, 100.0) | .0319† | |
| ALT, mg/L** | 37.2 (5, 213) | 48.2 (5, 213) | 31.1 (5, 79) | .0170† |
| Parameter . | All patients (n = 69; 42 female) . | Severe (n = 28; 17 female) . | Moderate (n = 41; 25 female) . | P for difference between moderate/severe . | Local controls (n = 25) . |
|---|---|---|---|---|---|
| Age, years* | 26.6 (3-61) | 17.5 (3-28) | 32.8 (9-61) | <.0001† | 16.6 (16.0, 17.2) |
| Hb, g/dL* | 6.2 (4.4-8.3) | 5.8 (4.4-7.6) | 6.4 (4.9-8.3) | .1397 | 15.0 (14.5, 15.5)‡ |
| HbF, %* | 26.0 (6.2-51.2) | 24.1 (6.2-42.4) | 27.3 (6.4-51.2) | .3226 | 0.27 (0.19, 0.39)‡ |
| Total transfusions* | 47.6 (0, 143) | 59.3 (3, 143) | 39.7 (0, 123) | .0377† | |
| Ferritin, mg/L** | 947 (143, 9790) | 1356 (328, 9790) | 732 (143, 3260) | .0021† | 48.2 (38, 61.2)‡ |
| Liver iron, mg/gdwt** | 7.21 (0.7, 54.2) | 11.3 (1, 54.2) | 5.30 (0.7, 29.0) | .0006† | |
| NTBI, µM/L* | 4.68 (−2.67, 23.75) | 5.16 (−1.67, 10.27) | 4.36 (−2.67, 23.75) | .4456 | |
| Hepcidin, ng/mL** | 2.06 (0.1, 51.8) | 2.24 (0.1, 51.8) | 1.95 (0.1, 36.3) | .7441 | 28.23 (18.83, 42.3)‡ |
| sTfR, nM/L** | 7.83 (1, 35.3) | 6.51 (1, 35.3) | 8.87 (1.4, 35.3) | .1472 | |
| EPO, mg/L** | 158 (28.7, 2196.7) | 159 (28.7, 2196.7) | 158 (34.5, 1296.5) | .9915 | |
| CRP, mg/L** | 1.61 (0.20, 34.23) | 1.66 (0.23, 34.23) | 1.58 (0.20, 13.01) | .8433 | |
| GDF-15, pg/mL,** | 8407 (1920, 60 229) | 7011 (2125, 19 031) | 9517 (1920, 60 229) | .0549 | |
| Labile plasma iron, µM/L* | 4.01 (−1.07, 13.61) | 3.60 (0.1, 7.7) | 4.29 (−1.07, 13.61) | .9320 | |
| Transferrin SAT, %* | 90.9 (31.9, 100.0) | 97.3 (48.3, 100.0) | 86.6 (31.9, 100.0) | .0319† | |
| ALT, mg/L** | 37.2 (5, 213) | 48.2 (5, 213) | 31.1 (5, 79) | .0170† |