How I treat thalassemia

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How I treat thalassemia by Eliezer A. Rachmilewitz, and Patricia J. Giardina Blood Volume 118(13):3479-3488 September 29, 2011 ©2011 by American Society of Hematology

Mechanism of IE and hemolysis in thalassemia. Eliezer A. Rachmilewitz, and Patricia J. Giardina Blood 2011;118:3479-3488 ©2011 by American Society of Hematology

Amelioration of free iron species (LPI and LCI) by iron chelators and antioxidants. Amelioration of free iron species (LPI and LCI) by iron chelators and antioxidants. Labile plasma iron (LPI) is penetrating through the cell membrane with a consequent accumulation of labile cell iron (LCI). Both LPI and LCI react with reactive oxygen intermediate (ROI) producing noxious reactive oxygen species (ROS), for example, OH' radicals, which are highly reactive and oxidize DNA, proteins and lipid components of the cell. Deferiprone (DFP) chelates LCI alone or in combination with LPI by Deferiozamine (DFO). Deferasirox (DFX) mainly removes LPI. Eliezer A. Rachmilewitz, and Patricia J. Giardina Blood 2011;118:3479-3488 ©2011 by American Society of Hematology