Abstract
After birth, fetal haemoglobin (Hb F), which consists of two α and two γ globins chains (α2,γ2), is normally replaced by adult haemoglobin (Hb A), which has two α and two β chains. Babies with homozygous β thalassaemia, who have defective β-chain synthesis, become anaemic during the first year of life. 1 Weatherall DJ Clegg JB The thalassaemia syndromes. Blackwell Scientific Publications, Oxford1981 Google Scholar Because treatment is limited to red-cell transfusions and iron chelation, or marrow transplantation, if feasible, pharmacological stimulation of Hb F production is being explored, although the results of clinical trials have been disappointing. 2 Olivieri NF Reactivation of fetal haemoglobin in patients with thalassaemia. Sem Hematol. 1996; 33: 24-42 PubMed Google Scholar We report a remarkable response to pharmacological treatment in two siblings with thalassaemia major.
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