Norbert Gattermann1. 1. Klinik für Hämatologie, Onkologie und klinische Immunologie, Heinrich-Heine-Universität Düsseldorf, Düsseldorf, Germany. Gattermann@med.uni-duesseldorf.de
Abstract
BACKGROUND: Because secondary hemochromatosis is due to hereditary or acquired anemia, phlebotomy is not a suitable means of removing excess iron in this situation. Rather, the treatment is based on the targeted elimination of iron by means of iron chelators. METHODS: Selective review of the literature. RESULTS: Disorders causing secondary hemochromatosis (e.g., thalassemia) are characterized by ineffective erythropoiesis leading to increased duodenal uptake of iron. Most patients are also chronically transfusion-dependent and receive 200-250 mg of iron with each transfused unit of packed red blood cells. As the excess iron cannot be actively excreted, iron overload ensues, which can cause organ damage. Most patients with this condition in Germany are elderly persons with myelodysplastic syndromes (MDS). The standard treatment to date, parenterally administered deferoxamine, is often hampered by poor compliance. In September 2006, a new oral iron chelator, deferasirox, was approved for use in Germany. According to current findings, this medication is safe, except for a low risk of renal or hepatic failure. CONCLUSIONS: Iron chelation is a treatment option not only for thalassemia patients, but also for those with lower-risk MDS who can be expected to need several years of transfusion therapy.
BACKGROUND: Because secondary hemochromatosis is due to hereditary or acquired anemia, phlebotomy is not a suitable means of removing excess iron in this situation. Rather, the treatment is based on the targeted elimination of iron by means of iron chelators. METHODS: Selective review of the literature. RESULTS: Disorders causing secondary hemochromatosis (e.g., thalassemia) are characterized by ineffective erythropoiesis leading to increased duodenal uptake of iron. Most patients are also chronically transfusion-dependent and receive 200-250 mg of iron with each transfused unit of packed red blood cells. As the excess iron cannot be actively excreted, iron overload ensues, which can cause organ damage. Most patients with this condition in Germany are elderly persons with myelodysplastic syndromes (MDS). The standard treatment to date, parenterally administered deferoxamine, is often hampered by poor compliance. In September 2006, a new oral iron chelator, deferasirox, was approved for use in Germany. According to current findings, this medication is safe, except for a low risk of renal or hepatic failure. CONCLUSIONS:Iron chelation is a treatment option not only for thalassemiapatients, but also for those with lower-risk MDS who can be expected to need several years of transfusion therapy.
Authors: Peter D Jensen; Finn T Jensen; Thorkil Christensen; Hans Eiskjaer; Ulrik Baandrup; Johan L Nielsen Journal: Blood Date: 2003-02-06 Impact factor: 22.113
Authors: Erik R Anderson; Matthew Taylor; Xiang Xue; Sadeesh K Ramakrishnan; Angelical Martin; Liwei Xie; Bryce X Bredell; Sara Gardenghi; Stefano Rivella; Yatrik M Shah Journal: Proc Natl Acad Sci U S A Date: 2013-11-26 Impact factor: 11.205