Table 1.
Clinical variables of the study population
| Clinical variables | Normal HbAA (Range; N=5) | HbSC (Mean ± SD; N=7) | HbSS Low OI (Mean ± SD; N=9) | HbSS High OI (Mean ± SD; N=9) | P-valueϯ |
|---|---|---|---|---|---|
| Occlusion Index (%) | 0.13–0.23 | 0.52 ± 0.15 | 0.93 ± 0.32 | 3.11 ± 0.60 | <0.001* |
| Age | N/A | 39 ± 7 | 39 ± 10 | 33 ± 9 | 0.233** |
| Hematocrit (%) | 36-50 | 32.3 ± 2.8 | 25.0 ± 3.75 | 23.4 ± 4.8 | 0.446* |
| Hemoglobin (g/dL) | 12–18 | 12 ± 1 | 9.2 ± 0.9 | 8.0 ± 1.3 | 0.037* |
| MCV (fL) | 82–95 | 82 ± 5 | 113 ± 12 | 92 ± 8 | 0.001* |
| WBC Count (109/L) | 4–11 | 11.5 ± 2.4 | 9.0 ± 3.2 | 10.1 ± 1.9 | 0.336* |
| Platelet Count (109/L) | 150–400 | 314 ± 84 | 389 ± 152 | 351 ± 108 | 0.552* |
| ANC (106/L) | 1500–8000 | 6896 ± 2600 | 5354 ± 2347 | 5482 ± 2012 | 0.903* |
| Reticulocyte (109/L) | 20–150 | 183 ± 54 | 174 ± 98 | 356 ± 86 | 0.001* |
| LDH (U/L) | 140–280 | 256 ± 194 | 222 ± 33 | 451 ± 126 | <0.001* |
| Ferritin (μg/L) | 12–300 | 182 ± 159 | 1218 ± 1572 | 544 ± 512 | 0.289** |
| Hemoglobin S (%) | N/A | 43.5 ± 7.3 | 71.3 ± 13.9 | 64.1 ± 30.2 | 0.659** |
| Hemoglobin A (%) | 99–100 | 6.2 ± 13.1 | 6.9 ± 13.5 | 24.9 ± 27.1 | 0.791** |
| Hemoglobin F (%) | 0–0.9 | 1.0 ± 1.1 | 17.6 ± 4.7 | 3.5 ± 2.9 | <0.001** |
| Diagnosis of Intrapulmonary Shunting | N/A | 0% of Subjects (0/7) | 44% of Subjects (4/9) | 89% of Subjects (8/9) | 0.046 (χ2) |
| On-hydroxyurea | N/A | 0% of Subjects (0/7) | 89% of Subjects (8/9) | 44% of Subjects (4/9) | 0.046 (χ2) |
A total of 30 blood samples were obtained from 5 healthy donors (HbAA), 7 subjects with hemoglobin-SC disease (HbSC), and 18 subjects with homozygous SCD (HbSS).
ϯ
: P-value for comparing the Low OI group and the High OI group.
*
: Parametric one-way ANOVA.
**
: Non-parametric Mann-Whitney U test.
χ2: Chi-squared test.
SD: Standard deviation.