Due to the variable phenotype of HbH, some people may develop transfusion dependence later in life despite a benign history in childhood. Additional manifestations including iron overload, osteopenia, splenomegaly, and biliary disease may still occur among non-transfusion dependent patients and in those with deletional disease [48]. This raises the question of whether these patients should pursue transplant in childhood in pursuit for a better prognosis.

From: Chapter 12, CURATIVE THERAPIES FOR α-THALASSAEMIA

Cover of Guidelines for the Management of α-Thalassaemia
Guidelines for the Management of α-Thalassaemia [Internet].
Amid A, Lal A, Coates TD, et al., editors.
Nicosia (Cyprus): Thalassaemia International Federation; 2023.
© Thalassaemia International Federation.

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