Table 1.
Hematological parameters and genotypes of 17 carriers of Hb Hekinan II with α-thalassemia and hemoglobinopathies (values are presented as mean ± SD or as raw data where appropriate).
| Parameters | Hb Hekinan II/α0-thalassemia | Hb Hekinan II/α+-thalassemia | Hb Hekinan II/Hb CS | Hb Hekinan II/Hb CS/Hb E | Hb Hekinan II/Hb CS/Hb EE | ||
|---|---|---|---|---|---|---|---|
| Number | 8 | 5 | 1 | 1 | 1 | 1 | |
| Age (year) | 23–72 (37.5 ± 16.0) |
3–41 (24.4 ± 14.6) |
25 | 76 | 32 | 21 | |
| RBC (x1012/L) | 5.99 ± 0.7 | 5.43 ± 0.58 | 6.16 | 4.61 | 5.05 | 5.84 | |
| Hb (g/dL) | 13.2 ± 2.0 | 13.9 ± 1.60 | 15.3 | 12.5 | 13.1 | 12.1 | |
| HCT (%) | 41.3 ± 5.2 | 43.5 ± 4.52 | 48.3 | 38.4 | 40.6 | 38 | |
| MCV (fL) | 67.9 ± 2.5 | 80.3 ± 7.50 | 78.0 | 83.5 | 80.3 | 64.2 | |
| MCH (pg) | 21.4 ± 1.1 | 25.6 ± 2.43 | 24.8 | 27.3 | 26.0 | 20.7 | |
| MCHC (g/dL) | 31.9 ± 1.6 | 31.8 ± 0.57 | 31.6 | 32.6 | 32.4 | 32.3 | |
| RDW-CV (%) | 15.1 ± 1.2 | 13.9 ± 0.40 | 14.9 | 16.1 | 17.0 | 16 | |
| Hb profilea | A2A with Hb Hekinan II | A2A | CSA2A | CSEA | EA | EE | |
| Hb A (%)a | 62.4 ± 1.7 | 86.1 ± 1.27 | 87.7 | 68.1 | 67.3 | 0.0 | |
| Hb A2 (%)a | 1.96 ± 0.3 | 2.30 ± 0.23 | 1.8 | na | na | na | |
| Hb A2+E (%)a | 0 | 0 | 0 | 15.8 | 22.2 | 86.0 | |
| Hb F (%)a | 1.0 ± 1.5 | 1.10 ± 1.56 | 2.4 | 0.9 | 1.0 | 1.5 | |
| Hb Hekinan II (%)a | 24.6 ± 1.6 | 0.0 | 0.0 | 0.0 | 0.0 | 0.0 | |
| Hb CS (%)a | 0.0 | 0.0 | 0.5 | 0.5 | 0.0 | 0.0 | |
| α-globin genotype | ααHekinan/–SEA | ααHekinan/-α3.7 | ααHekinan/αCSα | ααHekinan/αCSα | ααHekinan/αCSα | ||
| β-globin genotype | βA/βA | βA/βA | βA/βA | βA/βE | βE/βE | ||
RBC, Red Blood Cell; Hb, Hemoglobin; HCT, Hematocrit; MCV, Mean Corpuscular Volume; MCH, Mean Corpuscular Hemoglobin; MCHC, Mean Corpuscular Hemoglobin Concentration; RDW-CV, Coefficient of Variation of the Red Cell Distribution Width; CS, Constant Spring; na, not available.
aDetermined using high performance liquid chromatography (HPLC) (Variant II, Bio-Rad Laboratories).