Combination Iron Chelation Therapy with Deferiprone and Deferasirox in Iron-Overloaded Patients with Transfusiondependent β-thalassemia Major

Abstract

There are few papers on the combination therapy of deferiprone (DFP) and deferasirox (DFX) in iron-overloaded patients with transfusion-dependent β-thalassemia major (β-TM). A total of 6 patients with β-TM (5 males and 1 female) with a mean age of 23.8±5.8 years (ranging from 17 to 31) used this treatment regimen. The mean doses of DFP and DFX were 53.9±22.2 and 29.3±6.8 mg/kg/day, respectively. The duration of treatment was 11.5±4.6 months. Their serum ferritin levels were measured to be 2800±1900 and 3400±1600 ng/mL before and after treatment, respectively (p<0.6). Their cardiac magnetic resonance imaging (MRI) T2* values were 16.69±15.35 vs 17.38±5.74 millisecond (ms) before and after treatment, respectively (p < 0.9). Although there was no significant difference between their cardiac MRI T2* values before and after treatment statistically, the values improved after combination therapy with DFP and DFX in most of the patients. Liver MRI T2 * values were changed from 2.12±0.98 to 3.03±1.51 ms after treatment (p < 0.01); Further, their liver T2* values and liver iron concentration (LIC) were improved after treatment. Our study found that cardiac MRI T2* values, liver MRI T2* values, and LIC were improved after combination therapy with DFP and DFX in β-TM patients and that DFP and DFX combination therapy could be used to alleviate cardiac and liver iron loading.

Iron‐chelating effect of silymarin in patients with β‐thalassemia major: A crossover randomised control trial

“…MRI indexes have been used as one of the most important indicators for evaluating the iron burden in β‐TM patients (Voskaridou et al, ). The MRI‐based method for determining the LIC is a highly sensitive and specific method that reflects body iron store (Karami, Kosaryan, Amree, Darvishi‐Khezri, & Mousavi, ). Studies revealed that there is stronger correlation between LIC and body iron loading in patients with β‐TM (Voskaridou et al, ).…”

Administration of Intravenous Zoledronic Acid Every 3 Months vs. Annually in beta-thalassemia Patients with Low Bone Mineral Density: a Retrospective Comparison of Efficacy

“…During the case enrollment process, patient files were reviewed to obtain data on the following: lumbar spine and femoral neck z -scores at annual BMD measurements, age, gender, dependent/not dependent on blood transfusions, duration of blood transfusion(s), units of transfused blood per year, received iron via blood transfusion, iron-chelating therapy data, mean serum ferritin and hemoglobin (measured during the previous five years), mean ca (mg/dL) and vitamin D (ng/mL) levels during treatment with zoledronic acid, history of splenectomy, and history of diabetes mellitus. Each PRBC (packed red blood cell) packed contains 250 mg of iron; so, the iron intake from blood transfusions in mg/year was calculated as: 250 × the number of units given during one year ( 21 ). All patients were on a steady dose of iron-chelators during treatment with zoledronic acid [desferrioxamine (20-40 mg/kg/day), deferasirox (20-40 mg/kg/day), and deferiprone (50-100 mg/kg /day)].…”

Deferasirox: Over a Decade of Experience in Thalassemia

“…Karami et al reported results of combining DFP (mean dose of 53.9±22.2 mg/kg/d) with DFX (mean dose of 29.3±6.8 mg/kg/d) in 6 patients with TDT after failing monotherapy, and showed non-significant increases in SF with a significant effect on LIC (change by 7.59±3.16 mg/g dw). 67 Furthermore, 33 patients with TDT who failed monotherapy were evaluated in a prospective study at a single center in India, with 12 patients continuing the 2-year treatment with 75 to 100 mg/kg/d DFP (divided into three doses) and 20 to 40 mg/kg/d DFX. 68 This regimen showed a reduction in the mean SF by 44.67%±13.78% at two years and was well tolerated with GI disturbances noted in around 6% of patients, and elevations in creatinine >33% above the baseline on two consecutive occasions noted in around 85% of patients.…”