Results of long-term iron-chelating therapy.
The improvement in survival and quality of life of iron-overloaded patients achieved by regular subcutaneous chelation has been extensively documented over the years. A review of the long-term results allows one to establish the following points: (1) with regular subcutaneous chelation, a negative iron balance can be obtained in most patients, except very young ones; (2) severe deferoxamine (DFO) toxicity may be prevented by skipping high doses and by carefully monitoring and modulating chelation, especially in patients with a low iron overload; (3) the maintenance of compliance with DFO over 0.6 and of ferritin levels below 2,000 prevents iron overloaded complications, at least for the first 20 years of life; (4) long-term chelation can reverse functional complications such as liver fibrosis, arrhythmia and echocardiographic abnormalities, but not complications due to extensive tissue alterations, such as frank diabetes, hypothyroidism and myocardiosclerosis; (5) intensive intravenous protocols can be successfully applied in heavily overloaded patients and represent the only possibility to reverse their dangerous iron burden in a relatively short period of time; (6) survival and quality of life in well-chelated patients are approaching a normal pattern, and (7) clinical outcome and prognosis are better evaluated by parameters that consider iron overload and chelation trends.
Gabutti
V
V
Paediatric Department, University of Turin, Italy.
Piga
A
A
D000293:Adolescent; D000328:Adult; D003676:Deferoxamine / Q000009:adverse effects / Q000627:therapeutic use; D005260:Female; D005293:Ferritins / Q000097:blood; D006486:Hemosiderosis / Q000188:drug therapy* / Q000209:etiology; D006801:Humans; D007502:Iron Chelating Agents / Q000008:administration & dosage / Q000009:adverse effects* / Q000627:therapeutic use*; D008297:Male; D013789:Thalassemia / Q000628:therapy; D065227:Transfusion Reaction
D007502:Iron Chelating Agents; D005293:Ferritins; D003676:Deferoxamine