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Table 2 Characteristics and complications in NTDT patients*[5],[16],[20],[35]-[41]

From: Identification and key management of non-transfusion-dependent thalassaemia patients: not a rare but potentially under-recognised condition

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 +++ + +
  1. *Viprakasit V, unpublished data.
  2. β-thalassaemia syndromes include deletional HbH and non-deletional HbH disease.
  3. Frequency of complications are expressed as:
  4. 0-10%: +
  5. 10-30%: ++
  6. 30-60%: +++
  7. 60-100%: ++++.