title: [Immunologic studies in thalassemia major].
abstract: It is accepted that the immune alterations in patients with thalassemia major (TM) are secondary to the continuous transfusion-related antigenic stimulation together with iron overload. We evaluated the immune status of TM patients and found quantitative alterations in the distribution of peripheral blood lymphocyte subpopulations as well as functional alterations in natural killer (NK) cytotoxicity, B-cell differentiation, T-cell immunoregulation and phagocyte functional activities. TM patients, 10 years old or younger, have a lymphocyte profile and phagocytic activity similar to normal controls. Non-splenectomized thalassemic patients, older than 10, present lymphocytosis due to an increase in B lymphocytes and with splenectomy the T-CD8+ lymphocytes increase. With respect to phagocytes, the capacity to ingest candida is preserved while the candidacidal activity and the generation of toxic oxygen metabolites during the respiratory burst are diminished, and are inversely proportional with age and serum ferritin concentration, that is, older in age and higher in iron overload, more profound are the phagocyte dysfunctions. The altered B-cell function, the dysfunction of T immunoregulatory cells and the defective NK activity observed in TM patients were independent of the age of the patients and they were observed even in children younger than 10 years old and in general are attributed to blood transfusions. Moreover, there are some alterations that thalassemic carriers can express such as a defect at the level of NK and at B-cell function regulations, suggesting a possible genetic origin. Although complex, TM constitutes a human model that allows the dissection of specific immune defects, involving multiple factors, and can provide a better comprehension of how this complex immunoregulatory system works.
authors.forename: L
authors.initials: L
authors.lastname: Sen
authors.forename: M A
authors.initials: MA
authors.lastname: Goicoa
authors.forename: P J
authors.initials: PJ
authors.lastname: Nualart
authors.forename: I J
authors.initials: IJ
authors.lastname: Ballart
authors.forename: F
authors.initials: F
authors.lastname: Palacios
authors.forename: R A
authors.initials: RA
authors.lastname: Diez
authors.forename: M E
authors.initials: ME
authors.lastname: Estévez
mesh_terms: D006801:Humans; D007501:Iron / Q000378:metabolism; D007694:Killer Cells, Natural / Q000502:physiology; D007958:Leukocyte Count; D008214:Lymphocytes / Q000473:pathology*; D008264:Macrophages / Q000502:physiology; D009000:Monocytes / Q000502:physiology; D013156:Splenectomy / Q000009:adverse effects; D013789:Thalassemia / Q000276:immunology*; D065227:Transfusion Reaction
chemical_list: D007501:Iron
vernacular_title: Estudios inmunológicos en talasemia mayor.