1,721,143 research outputs found
Deferasirox Efficacy and Safety for the Treatment of Transfusion- Dependent Iron Overload in Patients with a Range of Rare Anemias
No full textIron Chelation Therapy with Deferasirox for Transfusional Iron-Overloaded Patients with Aplastic Anaemia and Other Rare Anaemias
No full textBlunted Serum Erythropoietin Response to Anemia in Patients Polytransfused for Beta-Thalassemia Major
No full textPurpose: To investigate the response of erythropoietin (EPO) to anemia in patients polytransfused for beta-thalassemia major.Patients and Methods: We measured the serum EPO levels and the concurrent hemoglobin (Hb) concentrations in 40 patients polytransfused for beta-thalassemia major, in 18 patients with iron deficiency anemia (IDA), and 32 healthy subjects. Serum EPO levels were assayed by an enzyme immunoassay.Results: in both groups with beta-thalassemia major and IDA, serum EPO levels were significantly elevated( 114 +/- 71 and 239 +/- 217 mU/mL, respectively). There was a significant inverse correlation between log EPO values and Hb concentrations in patients with beta-thalassemia major ( r = 0.61; p <0.01 ) and IDA (r = 0.81; p <0.01). In a semilogarithmic plot, the slope of the regression line obtained in patients with beta-thalassemia major was significantly lower than that of LDA (p <0.01), suggesting a blunted EPO response to anemia in patients polytransfused for beta-thalassemia major. The elevation of serum EPO in patients with beta-thalassemia major was poorly related to clinical Variables except serum ferritin.Conclusions: We conclude that a significant inverse relationship between serum EPO levels and Hb concentration exists in patients with beta-thalassemia major. However, this EPO response in patients with anemia caused by beta- thalassemia major may be blunted when compared to patients with IDA
Response of Iron Overload to Deferasirox in Rare Transfusion-Dependent Anaemias: Equivalent Effects on Serum Ferritin and Labile Plasma Iron for Haemolytic or Production Anaemias
No full textObjectives: It is widely assumed that, at matched transfusional iron- loading rates, responses to chelation therapy are similar, irrespective of the underlying condition. However, data are limited for rare transfusion- dependent anaemias, and it remains to be elucidated if response differs, depending on whether the anaemia has a primary haemolytic or production mechanism. Methods: The efficacy and safety of deferasirox (Exjade (R)) in rare transfusion-dependent anaemias were evaluated over 1 yr, with change in serum ferritin as the primary efficacy endpoint. Initial deferasirox doses were 10-30 mg/kg/d, depending on transfusion requirements; 34 patients had production anaemias, and 23 had haemolytic anaemias. Results : Patients with production anaemias or haemolytic anaemias had comparable transfusional iron-loading rates (0.31 vs. 0. 30 mL red blood cells/kg/d), mean deferasirox dosing (19.3 vs. 19.0 mg/kg/d) and baseline median serum ferritin (2926 vs. 2682 ng/mL). Baseline labile plasma iron (LPI) levels correlated significantly with the transfusional iron-loading rates and with serum ferritin levels in both cohorts. Reductions in median serum ferritin levels were initially faster in the production than the haemolytic anaemias, but at 1 yr, similar significant reductions of 940 and 617 ng/mL were attained, respectively (-26.0% overall). Mean LPI decreased significantly in patients with production (P < 0. 0001) and haemolytic (P = 0.037) anaemias after the first dose and was maintained at normal mean levels (< 0.4 mu m) subsequently. The most common drug- related, investigator- assessed adverse events were diarrhoea (n = 16) and nausea ( n = 12). Conclusions: At matched transfusional iron-loading rates, the responses of rare transfusion-dependent anaemias to deferasirox are similar at 1 yr, irrespective of the underlying pathogenic mechanism
Efficacy of Deferasirox in Patients with Thalassaemia Major Is Not Affected by a History of Hepatitis B or C
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