Prevention of Iron Overload and Long Term Maintenance of Normal Iron Stores in Thalassaemia Major Patients using Deferiprone or Deferiprone Deferoxamine Combination.
Decrease in mortality and morbidity is observed in thalassaemia major patients with reduced iron load in comparison to heavy iron loaded patients. Effective and complete treatment of transfusional iron overload can be achieved by chelation protocols that can eliminate excess iron and maintain normal iron stores (NIS). The maintenance of NIS, i. e., serum ferritin (350 μg/L >), MRI T2* cardiac (>20 ms) and liver (>6.3 ms) relaxation time levels was monitored in 16 thalassaemia major patients (32-53 years, 12 splenectomized, 10 male, erythrocyte transfusions 120-323 ml/kg/year) for about 90 patient years. The patients were treated with individualised tailor-made deferiprone or deferiprone/deferoxamine combination protocols. In 8 patients deferiprone (50-100 mg/kg/day) was sufficient for maintaining NIS and withdrawal of deferiprone for 28 months in total was necessary in 4 patients for preventing iron deficiency. In 3 other patients intermittent deferoxamine (50-75 mg/kg/8-30 h, 1-4 days/week) in combination with deferiprone (75-100 mg/kg/day) was sufficient for maintaining NIS. In the remaining 5 patients deferiprone (75-100 mg/kg/day) and deferoxamine (50-60 mg/kg/8-15 h, 1-7 days/week) combination was used for maintaining NIS, as a result of increased transfusions which were caused mainly by splenomegaly and infections. No toxic side effects were detected during the study. Lower chelation doses were used for the maintenance of NIS in comparison to iron loaded categories of patients. The safe maintenance of NIS using deferiprone and deferiprone/deferoxamine combinations should be considered as an optimum therapy for the complete treatment of iron overload in the majority of thalassaemia patients.
Postgraduate Research Institute of Science, Technology, Environment and Medicine Limassol, Cyprus.|Thalassaemia Unit, Paphos General Hospital, Paphos, Cyprus.
Annita
A
Kolnagou
Postgraduate Research Institute of Science, Technology, Environment and Medicine Limassol, Cyprus.
Christina N
CN
Kontoghiorghe
Postgraduate Research Institute of Science, Technology, Environment and Medicine Limassol, Cyprus.
George J
GJ
Kontoghiorghes
D000328:Adult; D000077543:Deferiprone; D003676:Deferoxamine / Q000627:therapeutic use*; D004359:Drug Therapy, Combination; D005260:Female; D005293:Ferritins / Q000097:blood; D006321:Heart / Q000000981:diagnostic imaging; D006801:Humans; D007501:Iron / Q000378:metabolism; D007502:Iron Chelating Agents / Q000627:therapeutic use; D019190:Iron Overload / Q000517:prevention & control*; D008099:Liver / Q000000981:diagnostic imaging / Q000378:metabolism; D008297:Male; D008875:Middle Aged; D009206:Myocardium / Q000378:metabolism; D011728:Pyridones / Q000627:therapeutic use*; D017086:beta-Thalassemia / Q000097:blood / Q000188:drug therapy*
D007502:Iron Chelating Agents; D011728:Pyridones; D000077543:Deferiprone; D005293:Ferritins; D007501:Iron; D003676:Deferoxamine