Blood Transfusion - 1 2010 (January - March)
Optimal response to thalidomide in a patient with thalassaemia major resistant to conventional therapy
Authors:  Nicoletta Masera, Luisa Tavecchia, Marietta Capra, Giovanni Cazzaniga, Chiara Vimercati, Lorena Pozzi, Andrea Biondi, Giuseppe Masera
Pages:  63-5
To cite this article:  Blood Transfus 2010; 8:63-5
Doi:  10.2450/2009.0102-09
Published online:  23/07/2009

Beta-Thalassaemia major is a hereditary anaemia, resulting from defects in β-globin production. The co-existence of hereditary persistence of foetal haemoglobin (HPFH) during adult life in patients with β-Thalassaemia reduces the severity of the disease; these patients have a mild disorder, sometimes not even requiring chronic transfusions. The clinical benefit of increasing HbF, first inferred in 19761, is due to a decrease in the imbalance between β and non-β-chains and the consequent reduction of haemolysis. Since then, many drugs have been studied as inducers of HbF for β-Thalassaemia and Sickle Cell Disease (SCD). Hydroxyurea (HU) is currently used in moderate to severe forms of SCD2,3 and in some cases of Thalassaemia intermedia4,5. ..
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