Chelation protocols for the elimination and prevention of iron overload in thalassaemia

Front Biosci (Landmark Ed). 2018 Jan 1;23(6):1082-1098. doi: 10.2741/4634.

Abstract

Iron overload toxicity is the main cause of mortality and morbidity in thalassaemia patients. The complete elimination and prevention of iron overload is the main aim of chelation therapy, which can be achieved by chelation protocols that can effectively remove excess iron load and maintain body iron at normal levels. Deferiprone and selected combinations with deferoxamine can be designed, adjusted and used effectively for removing all excess stored iron and for maintaining normal iron stores (NIS) in different categories of thalassaemia patients. High doses of deferiprone (75-100 mg/kg/day) and deferoxamine (50-60 mg/kg, 1-7 days/week) combinations can be used for achieving and maintaining NIS in heavily iron loaded transfused patients. In contrast, deferiprone (75-100 mg/kg/day) can be used effectively and sometimes intermittently for maintaining NIS in non heavily transfused patients. Deferasirox can in particular be used in patients not tolerating deferoxamine and deferiprone. The design of tailored made personalised protocols using deferiprone and selected combinations with deferoxamine should be considered as optimum chelation therapies for the complete treatment and the prevention of iron overload in thalassaemia.

MeSH terms

  • Benzoates / therapeutic use
  • Chelation Therapy / methods*
  • Deferasirox
  • Deferiprone
  • Deferoxamine / therapeutic use
  • Drug Therapy, Combination
  • Humans
  • Iron Chelating Agents / therapeutic use*
  • Iron Overload / complications
  • Iron Overload / drug therapy*
  • Iron Overload / prevention & control*
  • Pyridones / therapeutic use
  • Thalassemia / complications*
  • Triazoles / therapeutic use

Substances

  • Benzoates
  • Iron Chelating Agents
  • Pyridones
  • Triazoles
  • Deferiprone
  • Deferoxamine
  • Deferasirox