A decisional algorithm to start iron chelation in patients with beta thalassemia. [The specific aim of this study was to prospectively evaluate the appearance of LPI in children with thalassemia major and its correlation with the most commonly used parameters for determining the onset of chelation therapy, namely the number of transfusions that patients have undergone and the serum levels of ferritin, but also with the number of grams of administered red blood cells (RBCs) and with the percentage of transferrin saturation (TSAT)]. In non- chronically-transfused thalassemia patients, ferritin hepatosecretion does not match liver iron accumulation and neither is serum ferritin representative of extrahepatic siderosis. What are other plasma factors that might be more pertinent to the etiopathology of iron overload and relevant for ini- tiating treatment and assessing its long-term efficacy? Labile plasma iron (LPI), the major redox-active and readily chelatable fraction of non-trans- ferrin-bound iron (NTBI), is a more direct marker of impending tissue iron overload and, therefore, a potential indicator for the initiation of chelation therapy. This is based this consideration on the fact that detectable LPI levels (>0.2 μM) were found only in patients with transferrin saturation over 70%, that its components infiltrate cells by non-iron regulated routes causing labile cell iron to rise to toxic levels, and that they are direct targets of chelation in plasma as in cells.
Posted on: Fri, 14 Mar 2014 11:42:41 +0000
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