title: Immune and neural status of thalassemic patients receiving deferiprone or combined deferiprone and deferoxamine chelation treatment.
abstract: Deferiprone (L1), has previously been reported to be associated with immunological abnormalities in iron loaded thalassemia patients. However, other factors may also have similar effects such as the level of iron overload, chronic immuno-stimulation due to transfusions, splenectomy and deferoxamine (DFO). During chelation therapy with DFO, several complications have been reported, which were due to pharmacological activity and high dose toxicity with regard to both acoustic and visual effects, as well as peripheral nerve disorders that were measured by nerve conduction velocities. The immune and neural status of 44 beta-thalassemic patients, aged 10-30 years (mean 19.4 +/- 4.9), receiving L1 as a monotherapy (n = 21), or in combination with DFO (n = 23), has been followed for 2 years by monitoring the level of immunoglobulins (IgG, IgM, IgA), the level of T and B lymphocytes (CD4/CD8), the auto antibodies: anti nuclear (ANA), anti-double-stranded (anti-ds DNA), anti reticulin (anti-R1), anti-extra nuclear (anti-ENA), anti histone (anti-AHA), anti liver-kidney-muscle (anti-LKM), anti-smooth muscle (anti-SMA), anti-thyroid (anti-ATA), anti-mitochondrial (anti-AMA) antibodies and the C-reactive protein. The percentage of patients with disorders of the immune and nervous system concerned very few cases. None of our patients with pathological findings in their immunological or neurophysiological examinations presented any signs or symptoms of involvement of the immune or nervous system. Further advantages have been identified for the oral use of L1 and its combination with DFO, including synergistic efficacy and lower dosing with limited toxicity.
authors.affiliation: Thalassaemia Unit, Hippokratio General Hospital, Thessaloniki, Greece. natalpearl@yahoo.gr
authors.forename: Natalie
authors.initials: N
authors.lastname: Tourkantoni
authors.forename: Miranda
authors.initials: M
authors.lastname: Athanassiou-Metaxa
authors.forename: Dimitrios
authors.initials: D
authors.lastname: Zafiriou
authors.forename: Vassiliki
authors.initials: V
authors.lastname: Tzimouli
authors.forename: Marina
authors.initials: M
authors.lastname: Economou
authors.forename: Antonia
authors.initials: A
authors.lastname: Taparkou
authors.forename: Vassilios
authors.initials: V
authors.lastname: Perifanis
authors.forename: Florendia
authors.initials: F
authors.lastname: Kanakoudi-Tsakalidou
mesh_terms: D000293:Adolescent; D000328:Adult; D001323:Autoantibodies / Q000097:blood; D001402:B-Lymphocytes / Q000276:immunology; D015913:Chelation Therapy; D002648:Child; D000077543:Deferiprone; D003676:Deferoxamine / Q000008:administration & dosage / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D005071:Evoked Potentials; D005293:Ferritins / Q000097:blood; D006801:Humans; D007107:Immune System / Q000502:physiology*; D007155:Immunologic Factors / Q000097:blood; D007502:Iron Chelating Agents / Q000627:therapeutic use; D019190:Iron Overload / Q000188:drug therapy; D011728:Pyridones / Q000008:administration & dosage / Q000627:therapeutic use; D013601:T-Lymphocytes / Q000276:immunology; D017086:beta-Thalassemia / Q000188:drug therapy* / Q000276:immunology / Q000503:physiopathology
chemical_list: D001323:Autoantibodies; D007155:Immunologic Factors; D007502:Iron Chelating Agents; D011728:Pyridones; D000077543:Deferiprone; D005293:Ferritins; D003676:Deferoxamine