Table 1.
Characteristics | Patients (n=1,000) |
---|---|
Mean age ± SD, years | 23.9±15.4 |
Mean age at initial blood transfusion ± SD, years | |
All subjects | 5.9±10.8 |
Hb E/beta-thalassemia | 5.8±10.0 |
Beta-thalassemia major | 2.4±4.6 |
Other | 9.1±15.6 |
Mean pre-transfused Hb ±SD, g/dL | 8.1±1.4 |
Mean serum ferritin ± SD, ng/mL | 2,161±2,179 |
Gender, n (%) | |
Female | 551 (55.1) |
Male | 449 (44.9) |
Splenectomy, n (%) | |
No | 736 (73.6) |
Yes | 264 (26.4) |
Current iron chelation, n (%) | |
None | 62 (6.2) |
Deferoxamine monotherapy | 12 (1.2) |
Deferiprone monotherapy | 485 (48.5) |
Deferasirox monotherapy | 237 (23.7) |
Combined Deferoxamine + Deferiprone | 78 (7.8) |
Combined Deferoxamine + Deferasirox | 55 (5.5) |
Combined Deferiprone + Deferasirox | 71 (7.1) |
Phenotype group, n (%) | |
Hb E/beta-thalassemia | 738 (73.8) |
Beta-thalassemia major | 113 (11.3) |
Hb H disease with Hb CS | 65 (6.5) |
EABart’s disease† | 23 (2.3) |
EABart’s disease with HbCS‡ | 44 (4.4) |
EFBart’s disease§ | 3 (0.3) |
EFBart’s disease with HbCS¶ | 3 (0.3) |
Other | 11 (1.1) |
Abbreviation: Hb, hemoglobin; Hb CS, hemoglobin Constant Spring.
Compound heterozygous Hb H and heterozygous Hb E.
Compound heterozygous Hb H with Hb CS and heterozygous Hb E.
Compound heterozygous Hb H and homozygous Hb E.
Compound heterozygous Hb H with Hb CS and homozygous Hb E.