Total management of thalassaemia major.

ß-Thalassemia Major: Experience at King Fahad Hofuf Hospital, Al-Hassa, Saudi Arabia

“…Our management was not different from the standard treatment, which comprised blood transfusion, subcutaneous DF infusion and splenectomy whenever indicated. 4,15 Although it is best to treat ß-thalassemia major by regular and aggressive blood transfusion so as to promote normal physical growth, avoid skeletal deformity and control morbidity from hypoxia, 15 such an aggressive transfusion program is unfortunately often complicated by iron overload, especially if not accompanied by adequate iron chelation. 16 We could not undertake such a hypertransfusion regime because of noncompliance of our patients with DF infusion.…”

Effect of transfusional iron intake on response to chelation therapy in β-thalassemia major

“…Transfusional iron intake varies among patients with thalassemia as a result of differences in spleen size or previous splenectomy, the underlying molecular defect, and other factors. [2][3][4] However, to prevent excessive iron deposition in critical organs such as the heart and liver, chelator-induced iron excretion must at least balance the rate of ongoing transfusional iron accumulation. 1 Similar principles apply to sickle cell disease (SCD), myelodysplastic syndromes (MDS), and other disorders for which regular RBC transfusions are an important part of overall management.…”

Congenital dyserythropoietic anemia type I (CDA I): molecular genetics, clinical appearance, and prognosis based on long-term observation

“…25 Treatment was usually begun when plasma ferritin reached a serum concentration of 1000 g/L (1000 g/L). Age at initiation of deferoxamine therapy ranged from 7 to 35 years (median, 21 years).…”