Chelation therapy in beta-thalassemia: the benefits and limitations of desferrioxamine.

Abstract

In summary, long-term studies of DFO therapy in multiply-transfused patients with beta-thalassemia major have clearly shown it to be generally safe and effective. Appropriate use of DFO can remove excess iron, prevent iron-induced organ damage, and improve survival in thalassemia patients. Patients who begin treatment at a young age can be protected from the lethal complications of iron overload for at least two decades, but chelation therapy may not always prevent or ameliorate late growth failure and/or delayed or absent puberty. Those with iron damage to the heart and possibly other organs may experience stability or improvement in function with intense chelation. High-dose intravenous DFO produces a rapid decrease in hepatic iron content and improved cardiac function but can also cause severe toxicity, as can normal doses in patients with a low iron burden. Continuing studies of DFO are necessary to help further define its long-term efficacy and toxicity. In particular, significant attention should be paid to new strategies aimed at fostering improved compliance with its use.

• Authors: P J Giardina, R W Grady
• Journal: Seminars in hematology (Semin Hematol) Vol. 32 Issue 4 Pg. 304-12 (Oct 1995) ISSN: 0037-1963 [Print] United States
• PMID: 8560288 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, P.H.S., Review)

Chemical References

• Chelating Agents
• Iron
• Deferoxamine

Topics

• Adolescent
• Adult
• Chelating Agents (adverse effects, therapeutic use)
• Chelation Therapy (adverse effects)
• Child
• Deferoxamine (adverse effects, therapeutic use)
• Growth Disorders (etiology, prevention & control)
• Hearing Loss (chemically induced)
• Heart Diseases (etiology, mortality, prevention & control)
• Hemochromatosis (etiology, therapy)
• Hemosiderosis (etiology, therapy)
• Humans
• Iron
• Kidney Diseases (chemically induced)
• Lung Diseases (chemically induced)
• Thalassemia (complications, therapy)
• Transfusion Reaction
• Vision Disorders (chemically induced)