SCA patients: clinical and biological characteristics
| Values . | No. . | Percent . |
|---|---|---|
| Sex | ||
| F | 102 | 47 |
| M | 115 | 53 |
| G6PD | ||
| Deficiency | 21 | 11.5 |
| Normal | 161 | 88.5 |
| Alpha genes | ||
| 2 | 15 | 8 |
| 3 | 67 | 35.8 |
| 4 | 104 | 55.6 |
| 5 | 1 | 0.5 |
| α-Thalassemia | ||
| Absent | 105 | 56.1 |
| Present | 82 | 43.9 |
| Beta haplotype | ||
| Car/Car | 67 | 43.8 |
| Ben/Ben | 36 | 23.5 |
| Sen/Sen | 11 | 7.2 |
| Others | 39 | 25.5 |
| Parameters at baseline, median (Q1-Q3) | ||
| WBC count, ×109/L | 183 | 14.200 (10.750-17.850) |
| Neutrophil count, ×109/L | 171 | 5.320 (3.855-7.955) |
| Platelet count, ×109/L | 182 | 355.000 (282.500-434.000) |
| Hemoglobin level, g/dL | 185 | 7.8 (7.2-8.7) |
| Hematocrit, % | 179 | 23.4 (21.6-27) |
| MCV, flt | 178 | 80.35 (74.23-85.2) |
| Reticulocyte count, ×109/L | 173 | 286.200 (213.400-359.200) |
| HbF, % | 169 | 13.3 (8.1-20.7) |
| SpO2, % | 69 | 98 (97-100) |
| LDH, IU/L | 163 | 978 (760.5-1210) |
| Values . | No. . | Percent . |
|---|---|---|
| Sex | ||
| F | 102 | 47 |
| M | 115 | 53 |
| G6PD | ||
| Deficiency | 21 | 11.5 |
| Normal | 161 | 88.5 |
| Alpha genes | ||
| 2 | 15 | 8 |
| 3 | 67 | 35.8 |
| 4 | 104 | 55.6 |
| 5 | 1 | 0.5 |
| α-Thalassemia | ||
| Absent | 105 | 56.1 |
| Present | 82 | 43.9 |
| Beta haplotype | ||
| Car/Car | 67 | 43.8 |
| Ben/Ben | 36 | 23.5 |
| Sen/Sen | 11 | 7.2 |
| Others | 39 | 25.5 |
| Parameters at baseline, median (Q1-Q3) | ||
| WBC count, ×109/L | 183 | 14.200 (10.750-17.850) |
| Neutrophil count, ×109/L | 171 | 5.320 (3.855-7.955) |
| Platelet count, ×109/L | 182 | 355.000 (282.500-434.000) |
| Hemoglobin level, g/dL | 185 | 7.8 (7.2-8.7) |
| Hematocrit, % | 179 | 23.4 (21.6-27) |
| MCV, flt | 178 | 80.35 (74.23-85.2) |
| Reticulocyte count, ×109/L | 173 | 286.200 (213.400-359.200) |
| HbF, % | 169 | 13.3 (8.1-20.7) |
| SpO2, % | 69 | 98 (97-100) |
| LDH, IU/L | 163 | 978 (760.5-1210) |
Average biologic 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 NADP to NADPH, measured by ultraviolet spectrophotometry. LDH range for the institution: 135-225 IU/L.
HbF indicates fetal hemoglobin; LDH, lactate dehydrogenase; MCV, mean corpuscular volume; and SCA, sickle cell anemia.