Desferrioxamine-induced iron outputs in Cooley's anemia. Results and perspectives
Cantore, N.; Tortarolo, M.; Volpe, E.; Gonnella, F.; Valente, A.; Buonanno, G.
Haematologica 66(2): 196-207
1981
ISSN/ISBN: 0390-6078 PMID: 6791996 Document Number: 178853
Seven splenectomized, transfusion-dependent .beta.-thalassemic patients, 6-17 yr old, were subjected since 1976 to a hypertransfusion regimen. Thereafter they were treated with monthly 20 h DFO C, 1 g. Each patient received in additional trials 2 courses of i.v. DFO, 50 and 100 mg/kg + Vit C, 500 mg, and 2 further courses of i.v. DFO, 50 and 100 mg/kg at the completion of 2 wk of daily oral Vit C, 1 g. High amounts of Fe excretion with a good dose-response relationship were reached in each case only when i.v. ascorbic acid was added to both the 50 and 100 mg/kg DFO infusions. No substantial variations of Fe chelation were obtained in 5 of the patients by changing both the doses and the routes of ascorbic acid supplementation. Investigations performed both at monthly intervals and in conjunction with the continuous infusions of the highest DFO doses tested ruled out any acute or chronic drug-induced untoward side effects. A projected study of Fe chelation will be possible in 6 of the patients by combining i.v. DFO, 100 mg/kg, performed at intervals of 15 days, with daily i.m. DFO bolus injections, 20 mg/kg, in the aim of reaching a negative Fe balance, defined as excretion of more Fe than is received through blood transfusions.