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      Pathogenesis and management of iron toxicity in thalassemia.

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      Annals of the New York Academy of Sciences
      Wiley

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          Abstract

          In thalassemia major, iron overload is the joint outcome of multiple blood transfusions and an inappropriately increased iron absorption associated with ineffective erythropoiesis. Threshold values for iron toxicity are a liver iron concentration exceeding 440 mmoles/g dry weight, serum ferritin >2500 ng/mL, DFO urinary iron excretion >20 mg/day, and transferrin saturation >75%. The outpouring of catabolic iron that exceeds the iron-carrying capacity of transferrin results in the emergence of non-transferrin-bound iron (NTBI). NTBI is cleared preferentially by the liver and myocardium at a rate exceeding 200 times that of transferrin iron. NTBI catalyzes the formation of free radicals, resulting in oxidative stress and damage to mitochondria, lysosomes, lipid membranes, proteins, and DNA. The long-term consequences of iron toxicity, including cirrhosis, myocardiopathy, and endocrine disorders, are preventable and mostly reversible by effective iron chelation therapy. Recent technologic advances in the documentation of organ-specific siderosis and the improved efficiency of iron chelating programs resulted in a spectacular improvement in the prevention of iron-induced end-organ failure and improved survival in thalassemic patients.

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          Author and article information

          Journal
          Ann N Y Acad Sci
          Annals of the New York Academy of Sciences
          Wiley
          1749-6632
          0077-8923
          Aug 2010
          : 1202
          Affiliations
          [1 ] Department of Hematology, Shaare Zedek Medical Center, Jerusalem, Israel. hershko@szmc.org.il
          Article
          NYAS5544
          10.1111/j.1749-6632.2010.05544.x
          20712765
          970f4f5c-0e97-4b7a-ac21-88d6c13ac7bf
          History

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