Effective combination therapy of deferiprone and deferoxamine for the rapid clearance of excess cardiac IRON and the prevention of heart disease in thalassemia. The Protocol of the International Committee on Oral Chelators.
The International Committee on Oral Chelators (ICOC) combination therapy protocol involving the administration of deferiprone (L1) during the day (80-110 mg/kg/day) and deferoxamine (DFO) (40-60 mg/kg at least 3 days/week) during the night for 8-12 hours using a pump, or the whole 24 hours using an elastomeric pump infuser, has been tested in 11 thalassemia patients (seven males, four females) over a period of 9-28 months. The patients had variable serum ferritin levels (0.54-4.6 mg/L) and cardiac iron load ranging from normal to severe siderosis levels (MRI T2*: 4.7-45 ms). There was a substantial overall reduction in serum ferritin levels (0.17-2.16 mg/L) and normalization of cardiac iron (MRI T2* >20 ms) in all patients. In two patients with severe and moderate cardiac iron load range levels, cardiac iron normalization was achieved within 9-10 months. Two patients on L1 monotherapy (80-120 mg/kg/day) maintained normal range MRI T2* cardiac iron levels over the same period. The ICOC combination therapy protocol appears to be the most effective and least cumbersome form of chelation treatment for the rapid clearance of excess iron from the heart.
Kolnagou
Annita
A
Postgraduate Research Institute of Science, Technology, Environment and Medicine, Limassol, Cyprus.
Kontoghiorghes
George J
GJ
D000284:Administration, Oral; D009202:Cardiomyopathies / Q000188:drug therapy* / Q000209:etiology / Q000473:pathology / Q000517:prevention & control; D015913:Chelation Therapy / Q000379:methods; D002985:Clinical Protocols; D003131:Combined Modality Therapy; D000077543:Deferiprone; D003676:Deferoxamine / Q000008:administration & dosage / Q000627:therapeutic use*; D004334:Drug Administration Schedule; D004359:Drug Therapy, Combination; D005260:Female; D005293:Ferritins / Q000097:blood; D006333:Heart Failure / Q000188:drug therapy / Q000209:etiology; D006486:Hemosiderosis / Q000188:drug therapy* / Q000209:etiology; D006801:Humans; D015918:Infusion Pumps, Implantable; D007263:Infusions, Parenteral; D007501:Iron / Q000378:metabolism*; D007502:Iron Chelating Agents / Q000008:administration & dosage / Q000627:therapeutic use*; D008279:Magnetic Resonance Imaging; D008297:Male; D009206:Myocardium / Q000378:metabolism* / Q000473:pathology; D010349:Patient Compliance; D011728:Pyridones / Q000008:administration & dosage / Q000627:therapeutic use*; D065227:Transfusion Reaction; D017086:beta-Thalassemia / Q000150:complications / Q000188:drug therapy* / Q000628:therapy
D007502:Iron Chelating Agents; D011728:Pyridones; D000077543:Deferiprone; D005293:Ferritins; D007501:Iron; D003676:Deferoxamine