Characteristics and complications | β-thalassaemia intermedia | HbE/β-thalassaemia | α-thalassaemia syndromes‡ |
---|---|---|---|
Presenting age (years) | Usually >2 | Usually >2 | Usually >2 |
Presenting Hb level (g/dL) | 7-10 | 9-12 (mild) | 8-11 |
6 -7 (moderately severe) | |||
HbF(%) | 3-50, but can be up to 100 | 3-50, but can be up to 100 | Not raised, but HbH (β4) and Hb Barts (β4) present |
HbA2/HbE(%) | >3.5-4 | 30-40 | <2 |
Jaundice | + | +++ | ++++ |
Growth retardation | + | +++ | + |
Bone and skeletal abnormalities | + | +++ | ++++ |
Splenomegaly | ++++ | ++++ | ++ |
Leg ulcers | ++ | + | + |
Cholelithiasis | +++ | + | ++ |
Acute haemolytic episodes | + | ++ | +++ |
Thrombotic events | ++ | + | + |
Extramedullary haematopoiesis | ++ | + | + |
PHT | +++ | + | + |