Immunological evaluation of patients with beta-thalassemia major.
Abnormalities in the immune system and zinc homeostasis in patients with beta-thalassemia major (TM) have been reported. Since zinc ion is essential for the efficiency of the immune system and is required to induce biological activity to thymulin (Zn-FTS), a biochemically defined thymic hormone, we investigated the plasma levels of zinc and both active thymulin (Zn-FTS) and total zinc saturable thymulin (Zn-FTS+FTS) in 18 patients with TM aged between 2 and 31 years and 22 normal controls of the same age. Inhibitory molecules anti-thymulin and the distribution of lymphocyte subsets were also analyzed. Patients with TM presented significantly lowered plasma zinc and thymulin levels when compared to normal subjects. The significant enhancement of the active form of the hormone after zinc addition in vitro suggests that low thymulin values found in TM are due not to a thymic failure in synthesizing and secreting the thymic hormone, but a defect in zinc saturation of the hormone. An impairment of cell subset distribution was also demonstrated. This study shows that zinc and thymulin deficiency contribute to the complex mechanisms underlying immune dysfunction in TM.
Consolini
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Dipartimento di Medicina della Procreazione e dell'Età Evolutiva, Istituto di Clinica Pediatrica, Università di Pisa, Via Roma, 67, I-56100 Pisa, Italy. rita.consolini@clp.med.unipi.it
Calleri
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Legitimo
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Massei
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D000293:Adolescent; D000328:Adult; D018941:Antigens, CD19 / Q000032:analysis; D001323:Autoantibodies / Q000097:blood; D017252:CD3 Complex / Q000032:analysis; D015496:CD4-Positive T-Lymphocytes; D019002:CD56 Antigen / Q000032:analysis; D018414:CD8-Positive T-Lymphocytes; D002648:Child; D002675:Child, Preschool; D002712:Chlorides / Q000494:pharmacology; D005260:Female; D006801:Humans; D018655:Lymphocyte Count; D016131:Lymphocyte Subsets; D008214:Lymphocytes / Q000276:immunology; D008297:Male; D013935:Thymic Factor, Circulating / Q000032:analysis / Q000276:immunology; D015032:Zinc / Q000097:blood; D017967:Zinc Compounds / Q000494:pharmacology; D017086:beta-Thalassemia / Q000276:immunology*
D018941:Antigens, CD19; D001323:Autoantibodies; D017252:CD3 Complex; D019002:CD56 Antigen; D002712:Chlorides; D017967:Zinc Compounds; C016837:zinc chloride; D013935:Thymic Factor, Circulating; D015032:Zinc