Baseline hematology and demographic data in Hb E-β thalassemia patients with different clinical severity
. | Hb E-β thalassemia, n = 116 . | . | . | ||
|---|---|---|---|---|---|
| Parameters . | Mild, n = 33 . | Moderate, n = 18 . | Severe, n = 65 . | ||
| No. of males (%) | 17 (51) | 11 (61) | 26 (40) | ||
| Age, y | 9.46 ± 5.03 | 10.23 ± 4.78 | 8.06 ± 4.36 | ||
| Hb, g/dL | 8.69 ± 0.90 | 6.81 ± 1.22 | 6.07 ± 0.98 | ||
| Hct, % | 27.73 ± 2.63 | 22.96 ± 3.79 | 20.34 ± 2.94 | ||
| MCV, fL | 60.43 ± 6.22 | 61.87 ± 5.44 | 66.57 ± 8.23 | ||
| MCH, pg | 18.9 ± 2.70 | 19.22 ± 3.49 | 18.2 ± 2.97 | ||
| Retic, % | 5.7 ± 2.06 | 7.9 ± 2.19 | 10.4 ± 5.13 | ||
| RDW, % | 28.33 ± 3.18 | 32.57 ± 2.98 | 35.26 ± 4.44 | ||
| Hb F, g* | 4.42 ± 1.51 | 3.35 ± 0.59 | 2.97 ± 0.85 | ||
| Hepatomegaly (%) | 7 (21) | 9 (50) | 33 (51) | ||
| Splenomegaly (%) | 14 (42) | 12 (66.7) | 51 (78.5) | ||
| Hx transfusion (%) | 5 (15)† | 9 (50) | 60 (92)‡ | ||
| Splenectomy (%) | 2 (6)† | 1 (5.5) | 14 (21.5) | ||
. | Hb E-β thalassemia, n = 116 . | . | . | ||
|---|---|---|---|---|---|
| Parameters . | Mild, n = 33 . | Moderate, n = 18 . | Severe, n = 65 . | ||
| No. of males (%) | 17 (51) | 11 (61) | 26 (40) | ||
| Age, y | 9.46 ± 5.03 | 10.23 ± 4.78 | 8.06 ± 4.36 | ||
| Hb, g/dL | 8.69 ± 0.90 | 6.81 ± 1.22 | 6.07 ± 0.98 | ||
| Hct, % | 27.73 ± 2.63 | 22.96 ± 3.79 | 20.34 ± 2.94 | ||
| MCV, fL | 60.43 ± 6.22 | 61.87 ± 5.44 | 66.57 ± 8.23 | ||
| MCH, pg | 18.9 ± 2.70 | 19.22 ± 3.49 | 18.2 ± 2.97 | ||
| Retic, % | 5.7 ± 2.06 | 7.9 ± 2.19 | 10.4 ± 5.13 | ||
| RDW, % | 28.33 ± 3.18 | 32.57 ± 2.98 | 35.26 ± 4.44 | ||
| Hb F, g* | 4.42 ± 1.51 | 3.35 ± 0.59 | 2.97 ± 0.85 | ||
| Hepatomegaly (%) | 7 (21) | 9 (50) | 33 (51) | ||
| Splenomegaly (%) | 14 (42) | 12 (66.7) | 51 (78.5) | ||
| Hx transfusion (%) | 5 (15)† | 9 (50) | 60 (92)‡ | ||
| Splenectomy (%) | 2 (6)† | 1 (5.5) | 14 (21.5) | ||
All variances (mean ± standard deviation [SD]) were compared using either a 2-tailed student t test or the χ2 test as appropriate. P < .05 was taken to be statistically significant. Hx transfusion indicates that the patient's transfusion history includes occasional transfusion and regular transfusion.
Hb F levels (g) are calculated from the proportion of Hb F (%) from cellulose acetate electrophoresis and total hemoglobin levels. This result suggests a significant contribution of Hb F in ameliorating the clinical phenotype in the mild group (4.42 g vs 2.97 g in severe group, P < .05).
Although some patients received transfusion and/or even splenectomy (n = 2) from other hospitals before being referred to our center, our re-evaluation for their clinical severity indicated that they fell into the mild group.
Hematology data in 30 patients (46%) in the severe group who received high transfusion with iron chelation regimen were collected and averaged before the treatment started.