Immunologic Evaluation of Children with Homozygous Beta-Thalassemia Treated with Desferrioxamine

Abstract

In 15 children with thalassemia major (age 4–17 years) a detailed analysis of different immune functions was performed: phagocyte function, specific cellular immunity, humoral defense system. All patients had been subjected to a desferrioxamine therapy and a high transfusion regimen. Examination of neutrophil function included adherence, random migration, chemotaxis, killing of Escherichi coli and production of superoxide radical; these neutrophil functions were shown to be normal. In addition, lymphocyte proliferation in response to different lectins (phytohemagglutinin, concanavalin A, pokeweed mitogen) was identical in patients and controls. However, the number of circulating T-lymphocytes, helper T-cells and B-lymphocytes was increased in some patients. This phenomenon probably reflects an unspecific stimulation of the antibody-producing cells by repeated blood transfusions.

Decreased Phagolysosomal Fusion of Peripheral Blood Monocytes from Patients with Thalassemia Major

“…This is in accordance with other authors who showed that deferoxamine, at similar concentrations, en hanced the in vitro phagocytic function of human neutro phils [30,31]. Moreover, Speer et al [32] demonstrated that neutrophil function of children treated with deferox amine was similar to normal controls, suggesting that de feroxamine in vivo could improve neutrophil dysfunction, though they did not include in their study children without chelation therapy. In this study, the only TM patient who presented a normal value of phagolysosomal fusion was under continuous chelation therapy with deferoxamine since he initiated treatment with blood transfusions.…”

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.…”

“…Deferiprone, a recently adopted therapeutic alternative for iron-overloaded patients, has been associated with various immunological abnormalities [2][3][4][5]. However, relevant concerns have been raised against a background of immunologic dysfunction already reported in thalassemic patients, postulated to be secondary to iron overload, chronic immunostimulation due to transfusions, splenectomy and DFO action [6][7][8].…”

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

“…These abnormalities are ascribable to chronic immunostimulation due to transfusions, iron overload and the chelating treatment itself [10, 11, 12, 13, 14, 15]. …”