Abstract. Background: Beta thalassemia is an inherited hemoglobin disorder resulting in a severe, chronic anemia requiring life-long blood transfusion that induces iron overload. Silymarin is a flavonoid complex isolated from Silybin marianum with a strong antioxidant activity, inducing an hepatoprotective action, and probably, a protective effect on iron overload. The aim of this work was to determine the silymarin value in improving iron chelation in thalassemic patients with iron overload treated with Deferasirox. Patients and Methods: This study was conducted on 40 children with beta thalassemia major under follow-up at Hematology Unit, Pediatric Department, Tanta University Hospital with serum ferritin level more than 1000 ng/ml and was divided into two groups. Group IA: Received oral Deferasirox (Exjade) and silymarin for 6 months. Group IB: Received oral Deferasirox (Exjade) and placebo for 6 months and 20 healthy children serving as a control group in the period between April 2011 and August 2012 and was performed after approval from research ethical committee center in Tanta University Hospital and obtaining an informed written parental consent from all participants in this study. Results: Serum ferritin levels were markedly decreased in group IA cases compared with group IB (P= 0.001). Conclusion: From this study we concluded that, silymarin in combination with Exjade can be safely used in the treatment of iron-loaded thalassemic patients as it showed good iron chelation with no sign of toxicity. Recommendations: We recommend extensive multicenter studies in a large number of patients with longer duration of follow-up and more advanced techniques of assessment of iron status in order to clarify the exact role of silymarin in reducing iron overload in children with beta thalassemia.

BackgroundSickle cell disease has a worldwide distribution. Vaso-occlusive crisis (VOC) is one of the most important clinical features of the disease. Thrombospondin (TSP1) and Placenta growth factor (PlGF) have been reported to be involved in sickle cell diseases (SCD).ObjectiveThe aim of this study was to assess the clinical significance of Thrombospondin and Placenta growth factor profiles in patients with sickle cell disease.Patients and methodsThis study was carried out in sixty patients with sickle cell anemia who were attendants to Hematology units, Pediatric Departments, Tanta and Elmenofia University Hospitals in the period between December 2011 and May 2014 including thirty patients during vaso-occlusive crisis and thirty patients out of crisis. Also this study included twenty healthy children of matched age and sex as a control group. Serum TSP1 and PlGF levels were analyzed by ELISA.ResultsIn SCA patients with crisis the mean serum Thrombospondin level was 902.5±280.89 ng/mL; in SCA patients out of crisis the mean serum Thrombospondin level was 462.5 ± 190.2 ng/mL and in controls the mean value was 236.66±58.29 ng/mL. In SCA patients with crisis the mean serum Placenta growth factor level was 19.97±1.28 pg/ml; in SCA patients out of crisis the mean serum Placenta growth factor level was 13.12 ± 1.82 pg/ml and in controls the mean value was 9.89 ± 1.20 pg/ml. All paired comparisons for Thrombospondin and Placenta growth factor reached statistical significance (P< 0.001). There was significant positive correlation between serum Thrombospondin and Placenta growth factor levels in sickle cell anemia patients during crisis (r=0.848, p=<0.001).ConclusionsThis is the first study to show TSP1and PlGF concentration changes in patients with SCD in a large cohort study from Middle East, and to show correlation between both markers; therefore TSP1and PlGF may be useful VOC markers in SCD patients.RecommendationTo further assess TSP1 and PlGF as a marker of VOC in patients with SCD, further studies should be conducted to determine the exact point before VOC, when serum TSP1 and PIGF levels begin to increase. This requires monitoring of the TSP1 and PIGF levels in sickle cell patients out of crisis, showing how rapidly these levels increase just before VOC development.