Table 1.
Hematological data and clinical picture of subjects with HbE with different kinds of α-globin gene interactions
| Hb E | α-Globin gene | Hb (g/dl) | MCV (fl) | Hb typing | Hb E (%) | HbBart’s (%) | Hb F (%) | Clinical |
|---|---|---|---|---|---|---|---|---|
| Hb E heterozygote | αα/αα | 12.8 ± 1.5 | 84 ± 5 | EA | 29 ± 2.3 | - | 0.9 ± 0.7 | Normal |
| −α/αα | 13.1 ± 1.4 | 88 ± 4 | EA | 28 ± 1.5 | - | 0.7 ± 0.6 | Normal | |
| −/αα | 12.5 ± 1.4 | 77 ± 5 | EA | 21 ± 1.2 | - | 0.9 ± 0.4 | Normal | |
| −/−α | 9.1 ± 1.1 | 60 ± 3 | EFA Bart’s | 13 ± 2.1 | 4.5 ± 1.9 | 2.2 ± 1.9 | Thal intermedia (AEBart’s disease) | |
| Hb E homozygote | αα/αα | 10.6 ± 1.2 | 65 ± 3 | EF | 88 ± 2.6 | - | 3.6 ± 1.6 | Normal |
| −α/αα | 11.0 ± 1.6 | 65 ± 4 | EF | 87 ± 3.3 | - | 4.8 ± 3.7 | Normal | |
| −/αα | 10.5 ± 2.4 | 64 ± 7 | EF | 88 ± 5.7 | - | 3.8 ± 2.1 | Normal | |
| −/−α | 7.5 ± 0.8 | 60 ± 2 | EF Bart’s | 81 ± 1.5 | 4.2 ± 1.1 | 6.4 ± 1.2 | Thal intermedia (EFBart’s disease) | |
| Hb E β thalassemia | αα/αα | 7.1 ± 1.4 | 59 ± 3 | EF | 58 ± 9.5 | - | 38 ± 11.7 | Mild to severe disease |
| −α/αα | 8.5 ± 1.1 | 55 ± 3 | EF | 71 ± 7.5 | - | 24 ± 8.7 | Mild disease | |
| −/αα | 9.3 ± 0.5 | 52 ± 1 | EF | 84 ± 3.8 | - | 12 ± 2.5 | Mild disease | |
| −/−α | 7.6 ± 1.2 | 61 ± 2 | EF Bart’s | 82 ± 2.5 | 1.5 ± 0.3 | 5.5 ± 0.7 | Thal intermedia (EFBart’s disease) |