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%) |
Data missing from: a15 patients; b8 patients; c27 patients; and d29 patients