الفهرس 
BETA THALASSEMIIA
Systemic iron homeostasisOnly 14 pages are availabe for public view
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Abstract
Red blood cell transfusions have significantly increased the life expectancy of beta thalassemia major patients. However excess iron resulting from transfusion therapy override the capacity of transferrin, when TSAT is >70%, significant amounts NTBI appear in plasma (Brissot et al., 2012) and are deposited in cardiac myocytes, hepatocytes, pituitary cells, and pancreatic cells (Breuer et al., 2000).
Most of the guidelines recommend initiating iron chelation therapy after patients have received 10 or more transfusions or after reaching a serum ferritin level >1,000 ng/ml (Musallam et al., 2013).
In the current study we aimed to detect the time of appearance of LPI in infants with beta thalassemia major in relation to other markers of iron overload and to evaluate the efficacy and safety of the early usage of low dose deferiprone in thalassemia patients.
The current study was conducted on 37 beta thalassemia major patients over a period of 24 months from May 2015 to May 2017.We found that LPI levels positively correlated with TSAT levels (P<0.001) and age at enrollment (P=0.002), however there was no correlation between LPI and serum ferritin, number of transfusions, amount of transfused RBCs or transfusional iron loading rate.
LPI appearance was best predicted by TSAT (AUC=0.975), followed by number of units of transfused RBCs (AUC=0.79), and number of grams of transfused RBC (AUC=0.77), lastly was serum ferritin (AUC=0.66). The cut off value of each parameter was also determined (75% for transferrin saturation, 1090 grams of transfused RBCs, 6 times blood transfusion, and 500 ng/ml for serum ferritin).
After randomization all non-chelated patients reached end points prior to completing 12 months. With a mean follow up time 9.82 ± 2.71 versus 5.14 ± 2.18 months in chelated group. Serum ferritin was significantly higher in the non-chelated group at 3 month and 6 month visits (P= 0.005, P= 0.007). There was no difference in LPI level or TSAT between the two groups over the post randomization follow up period (P= 0.32, P= 0.27). Both groups had comparable adverse events (P=0.781).