Maintenance of normal range body iron store levels for up to 4.5 years in thalassemia major patients using deferiprone monotherapy.
New gold standard protocols are tested for the complete removal of iron overload in thalassemia using the International Committee on Chelation (ICOC) Maintaining Normal Body Iron combination protocol therapy of deferiprone (L1)/deferoxamine (DFO) and maintenance of normal range body iron store levels (NRBISL) using L1 monotherapy. Deferiprone (80-100 mg/kg/day) was administered for a mean of 21.3 months (range 7-91, total 171) in eight thalassemia major patients (four males and four females, 29-47 years) who have achieved serum ferritin (mean 108.5 microg/L, range 25-408), cardiac T2* (mean 31.5 ms, range 24-41) and liver T2* (30.1 ms, range 9.1-41) magnetic resonance imaging (MRI) relaxation times. At the end of the study normal range MRI T2* relaxation time was maintained in all eight patients with a cardiac mean of T2* 30.3 ms (range 23-37), liver T2* 28.8 ms (range 9.8-43) and serum ferritin with a mean of 173.7 microg/L (range 86-406). In one patient, this NRBISL was maintained for more than 4.5 years. No toxic side effects have been observed during the L1 monotherapy period.
Kolnagou
Annita
A
Postgraduate Research Institute of Science, Technology, Environment and Medicine, Limassol, Cyprus.
Kontoghiorghes
George J
GJ
D000328:Adult; D015913:Chelation Therapy / Q000379:methods*; D000077543:Deferiprone; D003676:Deferoxamine / Q000008:administration & dosage / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D007501:Iron / Q000378:metabolism*; D007502:Iron Chelating Agents / Q000008:administration & dosage / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D011728:Pyridones / Q000008:administration & dosage / Q000627:therapeutic use*; D013997:Time Factors; D017086:beta-Thalassemia / Q000188:drug therapy* / Q000378:metabolism*
D007502:Iron Chelating Agents; D011728:Pyridones; D000077543:Deferiprone; D007501:Iron; D003676:Deferoxamine