title: Chelation treatment in sickle-cell-anaemia: much ado about nothing?
abstract: Blood transfusions may prevent and treat serious complications related to sickle-cell disease (SCD) when performed according to specific guidelines. However, blood transfusion requirements in SCD inevitably lead to increased body iron burden. An adequate chelation treatment may prevent complications and reduce morbidity and mortality. This review evaluates the effectiveness, safety and costs of chelation treatment. The included trials were examined according to the recommendations of the American College of Cardiology (ACC) and the American Heart Association (AHA). Overall, 14 trials and a total of 502 patients with SCD were included in this review. Deferoxamine alone (s.c. or i.v.), deferiprone alone or versus deferoxamine, deferasirox versus deferoxamine and combined treatment with deferoxamine plus deferiprone were included and evaluated in the analysis. Only two randomized clinical trials have been reported. The results of this analysis suggest that use of chelation treatment in SCD to date has been based on little efficacy and safety evidence, although it is widely recommended and practised. The cost/benefit ratio has not been fully explored. Further research with larger randomized clinical trials needs to be performed.
authors.affiliation: U.O.C. Ematologia II con Talassemia, A.O. Villa Sofia-V. Cervello, Palermo Dipartimento di Scienze Statistiche e Matematiche S. Vianelli, Università di Palermo, Palermo U.O.S. Talassemia Pediatrica, A.O. Cardarelli, Napoli U.O.C. Medicina Trasfusionale, A.O. Villa Sofia-V. Cervello, Palermo, Italy.
authors.forename: Gaetano
authors.initials: G
authors.lastname: Lucania
authors.forename: Angela
authors.initials: A
authors.lastname: Vitrano
authors.forename: Aldo
authors.initials: A
authors.lastname: Filosa
authors.forename: Aurelio
authors.initials: A
authors.lastname: Maggio
mesh_terms: D000755:Anemia, Sickle Cell / Q000188:drug therapy*; D015913:Chelation Therapy; D003365:Costs and Cost Analysis; D064420:Drug-Related Side Effects and Adverse Reactions; D006801:Humans; D007502:Iron Chelating Agents / Q000009:adverse effects / Q000191:economics / Q000627:therapeutic use*; D016896:Treatment Outcome
chemical_list: D007502:Iron Chelating Agents