Effects of Deferiprone on Immune Status and Cytokine Pattern in Thalassaemia major

Abstract

Objective: The present study was undertaken to evaluate the possible occurrence of immunological abnormalities in thalassaemia major patients treated with deferiprone (L1). Methods: Longitudinal observational cohort study. Results: The absolute number of CD8+ lymphocytes was high and the CD4/CD8 ratio low before L1 treatment; these parameters returned to normal after 3 months of L1 treatment. TNF-α, IL-2 and IL-2sRα were elevated before L1 treatment (11.83 ± 1.75, 11.75 ± 3.91, 1,409 ± 621 pg/ml, respectively), while IL-6 was normal (2.58 ± 0.79 pg/ml). After 12 months of treatment, IL-10 was higher than in previous periods, although always within the normal range. TNF-α, IL-2 and IL-2sRα returned to normal after 12, 6, and 3 months of L1 treatment, respectively.

Immune Status of Thalassemic Patients Receiving Deferiprone or Combined Deferiprone and Desferrioxamine Chelation Treatment

“…Moreover, non-organ-specific antibody detection was relatively infrequent, with no coexistence of different autoantibodies in any patient. The increased immunoglobulin concentrations and lymphocyte counts found in the current study are also in agreement with other reports in thalassemic patients on L1 or DFO [8,9,11,12]. With respect to the advantages of oral chelation treatment as well as the suggested advantages of combined therapy, including synergistic efficiency and lower dosing with limited toxicity, our study supports the safety of L1 regarding immune function in thalassemic patients.…”

“…ANA and anti-dsDNA were Table 2. Autoantibodies present in the thalassemics before and after treatment also negative in both groups, a finding in accordance with other reports, indicating that the presence of these antibodies in L1-treated thalassemic patients is neither frequent nor associated with other parameters suggestive of autoimmune disease [9,10]. Moreover, non-organ-specific antibody detection was relatively infrequent, with no coexistence of different autoantibodies in any patient.…”

THE ROLE OF Treg CELLS AND FoxP3 EXPRESSION IN IMMUNITY OF β-THALASSEMIA MAJOR AND β-THALASSEMIA TRAIT PATIENTS

“…However, transfusion-dependent thalassemia major patients are under the effect of blood transfusions other than the recurrent infectious diseases, as a result of immunological abnormalities associated with the disease itself and/or treatment. Decrease in CD8 + T cells in thalassemia patients treated with deferiprone was shown by Del Vecchio et al [25]. This might be another explanation for increased CD8 + T cells percentage in thalassemia trait patients who never had iron chelation therapy when compared to thalassemia major patients treated with iron chelator agents.…”

The evaluation of carotid intima-media thickness in children with beta-thalassaemia major

“…[30][31][32][33][34] Patients with severe betathalassaemia major exhibit a pattern of altered immune response to antigenic stimulation related to transfusions and infectious agents, cytokine levels of stored allogenic blood, iron overload, and stroma cells of hyperplastic bone marrow. [35][36][37] The interplay between the iron overload, high oxidative stress, endothelial activation, proinflammatory state, and thalassaemic cells may contribute to the pathogenesis of atherosclerosis in these patients.…”