This protocol addresses the management of alpha thalassemia minor specifically in the context of alpha-thalassaemia major (haemoglobin Bart's hydrops foetalis). Infants with this condition who survive the neonatal period become lifelong transfusion-dependent, unless cured by haematopoietic stem cell transplantation.
Because of the distinct haematological picture in this population, the transfusion strategy is more aggressive than in other thalassaemia syndromes. A regular blood transfusion programme is the cornerstone of management — the specific parameters and defined thresholds are set out in the full protocol.
The primary aim is to maintain adequate functional haemoglobin. The protocol specifies the precise haemoglobin targets and monitoring criteria required to achieve and sustain this goal.
Once an infant with α-thalassaemia major (haemoglobin Bart's hydrops foetalis) survives the neonatal period, they will become lifelong transfusion-dependent, unless they are cured by haematopoietic stem cell transplantation.
However, patients with α-thalassaemia major require more aggressive transfusion strategies due to preserved reticulocytosis and high proportion of circulating non-functional HbH.
The goal of a transfusion regimen is to maintain functional haemoglobin (HbA) >90 g/L.
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