Transfusional hemosiderosis and combined chelation therapy in sickle thalassemia

Eur J Haematol. 2005 Oct;75(4):355-8. doi: 10.1111/j.1600-0609.2005.00528.x.

Abstract

Although the indications for transfusions in sickle cell syndromes are well listed, and chronic transfusion has become practicable since the recent advances in chelation therapy have essentially eliminated the risk of secondary iron overload, multi-transfused, non-compliant to long-term chelation therapy patients confront the complication of iron overload and secondary hemosiderosis. In thalassemia major patients, combined therapy with desferrioxamine and deferiprone has maximized tissue iron removal and may reduce the overall occurrence of hemosiderotic heart failure. Despite this, safety and contradictions of chelating agents are still controversial. The aim of this report is to present the results of this combination in a long-term transfused sickle beta-thalassemic patient suffering from severe heart failure and liver dysfunction.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Anemia, Sickle Cell / complications
  • Anemia, Sickle Cell / drug therapy
  • Anemia, Sickle Cell / therapy*
  • Blood Transfusion*
  • Chelation Therapy*
  • Deferiprone
  • Deferoxamine / therapeutic use
  • Drug Therapy, Combination
  • Heart Failure
  • Hemosiderosis / etiology
  • Hemosiderosis / prevention & control*
  • Humans
  • Liver Failure
  • Male
  • Pyridones / therapeutic use
  • beta-Thalassemia / complications
  • beta-Thalassemia / drug therapy
  • beta-Thalassemia / therapy*

Substances

  • Pyridones
  • Deferiprone
  • Deferoxamine