Table 1 Clinical characteristics of patients with transfusion dependent β-thalassaemia
Characteristic | N = 271 |
|---|---|
Mean (±SD) | |
Duration of the disease (years) | 9.8 (±3.8) |
Average pretransfusion haemoglobin (g/dL) | 8.4 (±1.1) |
Annual transfusion requirement (ml/kg/year) | 235 (±76) |
Average serum ferritin (ng/mL) | 1993 (±1844) |
Duration of hospital stay (days) | 2.29 (±1.0) |
Distance from home to hospital (km) | 54.8 (±48) |
Frequency (%) | |
Age groups | |
5–7 years | 68 (25.1%) |
8–12 years | 116 (42.8%) |
13–18 years | 87 (32.1%) |
Sub-type of thalassaemia | |
β-thalassaemia major | 228 (84.1%) |
HbE β-thalassaemia | 43 (15.9%) |
Thalassaemia treatment centre | |
Kurunegala | 155 (57.2%) |
Anuradhapura | 79 (29.1%) |
Ragama | 37 (13.7%) |
Frequency of blood transfusions | |
> 4 weekly | 29 (10.7%) |
4 weekly | 208 (76.8%) |
3 weekly | 33 (12.2%) |
< 3 weekly | 1 (0.4%) |
Average pretransfusion haemoglobin | |
< 7.0 g/dl | 32 (11.8%) |
7.0–8.9 g/dl | 132 (48.7%) |
9.0–10.5 g/dl | 99 (36.5%) |
> 10.5 g/dl | 8 (3.0%) |
Annual transfusion requirementa | |
< 200 ml/kg/year | 109 (42.6%) |
201–250 ml/kg/year | 41 (16.0%) |
251–300 ml/kg/year | 55 (21.5%) |
> 300 ml/kg/year | 51 (19.9%) |
Spleen status | |
No splenomegaly | 179 (66.1%) |
Splenomegaly of 1–3 cm | 74 (27.3%) |
Splenomegaly of > = 4 cm | 11 (4.1%) |
Splenectomised | 7 (2.5%) |
Liver status | |
No hepatomegaly | 188 (69.3%) |
Hepatomegaly of 1–2 cm | 67 (24.7%) |
Hepatomegaly > = 3 cm | 16 (5.9%) |
Serum Ferritinb | |
< 1000 ng/mL | 83 (31.6%) |
1001–2500 ng/mL | 122 (46.4%) |
2501–5000 ng/mL | 38 (14.4%) |
> 5000 ng/mL | 20 (7.6%) |
Iron chelator medication | |
No chelation | 1 (0.4%) |
Deferasirox | 163 (60.1%) |
Deferoxamine | 29 (10.7%) |
Deferiprone | 3 (1.1%) |
Deferasirox + Deferoxamine | 75 (27.7%) |
Complications | |
Thalassaemia facies | 92 (33.9%) |
Skin pigmentation | 57 (21.0%) |
Short staturec | 117 (48.0%) |
Undernutritiond | 85 (35.1%) |
Type 1 diabetes | 5 (1.8%) |
Hypothyroidism | 11 (4.1%) |
Cardiomyopathy | 2 (0.7%) |
Elevated transaminases | 58 (21.4%) |
Cirrhosis | 0 |
Allergic reaction to transfusion | 60 (22.1%) |
Hepatitis C infection | 60 (22.1%) |
Abdominal scars | 66 (24.4%) |
Hearing impairment | 4 (1.5%) |
Visual impairment | 19 (7.0%) |