Management of the thalassemias

Cold Spring Harb Perspect Med. 2013 Jun 1;3(6):a011767. doi: 10.1101/cshperspect.a011767.

Abstract

During the last 30 years, in addition to the considerable progress made in control and prevention of thalassemias(3), there have also been major advances in their symptomatic management, at least in wealthier countries where appropriate facilities are available. Remarkable improvements in survival in the severe forms of thalassemia have followed the more judicious use of blood transfusion and, in particular, the ability to manage the iron accumulation resulting from transfusion with its severe and ultimately lethal effects on endocrine and cardiac function.

Publication types

  • Review

MeSH terms

  • Benzoates / economics
  • Benzoates / therapeutic use
  • Blood Transfusion
  • Deferasirox
  • Deferiprone
  • Deferoxamine / economics
  • Deferoxamine / therapeutic use
  • Ferritins / blood
  • Humans
  • Iron / metabolism
  • Iron Chelating Agents / economics
  • Iron Chelating Agents / therapeutic use
  • Iron Overload / therapy
  • Liver / metabolism
  • Myocardium / metabolism
  • Pancreas / metabolism
  • Pituitary Gland, Anterior / metabolism
  • Pyridones / economics
  • Pyridones / therapeutic use
  • Thalassemia / therapy*
  • Triazoles / economics
  • Triazoles / therapeutic use

Substances

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