ABSTRACT
Background: Ongoing transfusional iron load (TIL) is an important determinant while deciding starting and subsequent dose adjustment of deferasirox during course of chelation therapy. So present study aims to find out effect of different dosing of deferasirox over the serum ferritin level in children with thalassemia major with impact of rate of transfusional iron load.Methods: This one year observational study was carried out in 35 transfusion dependent ?-thalassemic patients aged 2-18 years. Patients with baseline serum ferritin 1000-1500ng/ml and/or receiving TIL 0.2-0.3mg/kg/day were started 20mg/kg/day deferasirox and patients with ferritin>1500ng/ml and/or having TIL > 0.3mg/kg/day were started 30mg/kg/day deferasirox. Serum ferritin was repeated in every three months. Dose adjustments were performed on serum ferritin trends in steps of 5-10mg/kg /day to maximum 40mg/kg/day. Evaluation of relationship between dose adjustment, percentage of reduction in serum ferritin and TIL was done.Results: Group-1 patients(42.8%) had TIL 0.2 to 0.3mg/kg/day whereas Group-2(37.1%) and Group-3(20%) children had TIL >0.3-0.4mg/kg/day and >0.4 mg/kg/day respectively. Starting dose of deferasirox in 25.7% patients was 20mg/kg/day and in rest were 30mg/kg/day. Average dose of deferasirox in group-1 was significantly lower as compared to group-2 and group-3 patients ( p< 0.05). Significant decline in mean serum ferritin was observed in all three groups (p < 0.05). There was a significant positive correlation between TIL and average drug dose prescribed (r=0.5411and p=0.0007) but negative insignificant correlation was observed with percentage of reduction in serum ferritin(r=0.0027and p=0.98).Conclusions: Deferasirox 30mg/kg/day significantly reduces serum ferritin and is well tolerated in majority of patients having TIL 0.3-0.4mg/kg/day where as 20mg/kg/day is required in patients having low transfusional iron intake.
ABSTRACT
Purpose: Multi-organ hemosiderosis is a known complication in thalassemia patients with chronic blood transfusion. T2-Star (T2*) MRI has been introduced as a non-invasive tool for detecting iron overload in the liver and heart in these patients. This study is to determine and assess renal iron overload by MRI and its relation to liver and heart iron and serum ferritin in Iranian thalassemia patients. Methods: Total 821 transfusion dependent major and intermediate thalassemia patients (age range 10-50 years) were included in this study and calculations were done on their MRI data in a medical imaging center through 2014-2016. Iron values were calculated and averaged in a different region of interests (ROI) using fast-gradient-echo multi-echo T2* sequences. Results: Pathological renal iron content less than 36 ms was around 19.6%. The mean T2* kidney of the total population was 50.26 ms. A moderate negative, statistically significant correlation between kidney T2* relaxation time and serum ferritin was noted. For liver and heart, T2* relaxation time weakly, a statistically significant correlation was acquired by renal T2* relaxation time. Conclusions: Renal hemosiderosis was shown in numerous thalassemia patients. Since the frequency of renal iron deposition was approximately 20% in TM patients in a general population study, it might shed light that frequently monitors the renal iron loading merit hematologists in preventing the secondary side effects.
ABSTRACT
005), while iron overload occurred in 8 of 57 cases of ?-thalassemia (14.0%) and 89 out of 386 cases of ?-thalassemia (23.1%). Although the occurrence rate of iron overload in ?-thalassemia appeared higher than that in ?-thalassemia, no significant difference existed. Homozygote ?-thalassemia was prone to iron overload, followed by non-deletion HbH diseases, at the occurrence rate of 79.2% and 27.3% respectively. Iron deficiency was inclined to be associated with silent ?-thalassemia at the occurrence rate of 45.5%, followed by heterozygote ?-thalassemia (30.3%). Conclusion Both iron overload and iron deficiency can be found in thalassemia. Therefore, special treatments should be carried out according to given cases.