Iron Metabolism and Iron Chelators

Important advances in our understanding of the pathophysiology of the anemia of chronic disease have recently come from the laboratory of Drs. Elizabeta Nemeth and Tom Ganz and their colleagues at UCLA, and will be summarized at the Scientific Committee on Iron and Heme session (held today at 8:00 – 9:45 a.m. and 2:00 – 3:45 p.m.), chaired by Dr. Mark Fleming of Children’s Hospital of Boston. Hepcidin has been identified as an antimicrobial peptide that is regulated by iron, anemia, and inflammation, and results in hypoferremia via iron sequestration in the monocyte/macrophage system along with inhibitions of iron absorption from the gastrointestinal tract. Furthermore, a mutant hepcidin has been associated with severe juvenile hemochromatosis and may act in concert with the newlyidentified gene, hemojuvelin. Modifiers of hereditary hemochromatosis will be discussed by Dr. James Kushner, and hemojuvelin and juvenile hemochromatosis by Dr. Paul Goldberg in the Scientific Committee on Iron and Heme session, focusing on the clinical and research implications of these new proteins involved in iron metabolism.

New drugs to remove excessive iron, along with new methods for measuring tissue iron accumulation, are advancing two of the most challenging areas in the management of patients with thalassemia as well as other transfusion-dependent disorders. Dr. Renzo Galanello of the Universita di Cagliari will discuss recent advances in iron chelation therapy in the Education Session on Thalassemia (held 10:15 a.m. – 12:00 noon today and 7:30 – 9:15 a.m. tomorrow), chaired by Dr. Alan Cohen of Children’s Hospital of Philadelphia. Dr. Galanello will review the results of recent clinical trials assessing the safety and effectiveness of orally active chelators as well as novel forms of parenterally administered chelators.