Patients’ clinical and biological characteristics
| Characteristics . | SCA patients (n = 189) . |
|---|---|
| Sex | |
| Female | 101 (53.4%) |
| Male | 88 (46.6%) |
| G6PD | |
| Deficiency | 18 (10.6%) |
| Normal | 152 (88.8%) |
| α genes | |
| 2 | 15 (8.3%) |
| 3 | 61 (33.9%) |
| 4 | 102 (56.7%) |
| 5 | 2 (1.1%) |
| α-thalassemia | |
| Absent | 104 (57.8%) |
| Present | 76 (42.2%) |
| β haplotype | |
| Car/Car | 65 (39.6%) |
| Ben/Ben | 33 (20.1%) |
| Sen/Sen | 21 (12.8%) |
| Others | 45 (27.4%) |
| Parameters at baseline, n (median [Q1-Q3]) | |
| White blood cell count, 109/L | 171 (13.900 [11.400-17.200]) |
| Neutrophil count, 109/L | 164 (5.075 [4.015-7.372]) |
| Platelet count, 109/L | 169 (337.000 [269.500-438.000]) |
| Hemoglobin level, g/dL | 171 (8.1 [7.4-8.9]) |
| Hematocrit, % | 169 (24.6 [22.1-27.9]) |
| Mean corpuscular volume, fL | 168 (78.1 [71.6-85.1]) |
| Reticulocyte count, 109/L | 163 (273.700 [205.800-367.000]) |
| Fetal hemoglobin, % | 157 (15.0 [9.0-22.1]) |
| SpO2, % | 72 (98 [96-100]) |
| LDH, IU/L | 130 (732 [508-1028]) |
| Characteristics . | SCA patients (n = 189) . |
|---|---|
| Sex | |
| Female | 101 (53.4%) |
| Male | 88 (46.6%) |
| G6PD | |
| Deficiency | 18 (10.6%) |
| Normal | 152 (88.8%) |
| α genes | |
| 2 | 15 (8.3%) |
| 3 | 61 (33.9%) |
| 4 | 102 (56.7%) |
| 5 | 2 (1.1%) |
| α-thalassemia | |
| Absent | 104 (57.8%) |
| Present | 76 (42.2%) |
| β haplotype | |
| Car/Car | 65 (39.6%) |
| Ben/Ben | 33 (20.1%) |
| Sen/Sen | 21 (12.8%) |
| Others | 45 (27.4%) |
| Parameters at baseline, n (median [Q1-Q3]) | |
| White blood cell count, 109/L | 171 (13.900 [11.400-17.200]) |
| Neutrophil count, 109/L | 164 (5.075 [4.015-7.372]) |
| Platelet count, 109/L | 169 (337.000 [269.500-438.000]) |
| Hemoglobin level, g/dL | 171 (8.1 [7.4-8.9]) |
| Hematocrit, % | 169 (24.6 [22.1-27.9]) |
| Mean corpuscular volume, fL | 168 (78.1 [71.6-85.1]) |
| Reticulocyte count, 109/L | 163 (273.700 [205.800-367.000]) |
| Fetal hemoglobin, % | 157 (15.0 [9.0-22.1]) |
| SpO2, % | 72 (98 [96-100]) |
| LDH, IU/L | 130 (732 [508-1028]) |
Average biological parameters were obtained at baseline after the age of 12 months and before the age of 3 years, a minimum of 3 months away from a transfusion, 1 month from a painful episode, and before any intensive therapy (hydroxyurea, transfusion program, or stem cell transplantation). G6PD activity was assessed by reduction of NAD phosphate to reduced NAD phosphate, measured by UV spectrophotometry. LDH range for the institution was 135 to 225 IU/L.