title: Relative impact of HLA phenotype and CD4-CD8 ratios on the clinical expression of hemochromatosis.
abstract: Hemochromatosis is a hereditary iron-overload disease linked to HLA. The clinical expression of hemochromatosis is influenced by sex and age. However, other factors must account for the notorious heterogeneity of expression of the disease independent of sex, age, and HLA phenotype. The present study attempts to clarify some of these additional factors based on exhaustive statistical analysis of data collected from 43 selected patients with hemochromatosis. The statistical analysis focused on three groups of variables: the first group included variables reflecting the clinical expression of the disease; the second group represented the biochemical and hematological values at the time of diagnosis; and the third group consisted of the independent variables sex, age, HLA phenotype, and T-cell subset profile, i.e., the percentages and total numbers of CD4+ and CD8+ cells and the CD4-CD8 ratios. The results show that the relative expansion of the two main T-cell subsets, in the context of the HLA phenotype, correlates significantly with the clinical expression of hemochromatosis and the severity of iron overload. The present findings substantiate further the postulate that T cells have a role in the regulation of iron metabolism.
authors.affiliation: Department of Molecular Pathology and Immunology, Abel Salazar Institute for the Biomedical Sciences, Porto, Portugal.
authors.forename: G
authors.initials: G
authors.lastname: Porto
authors.forename: C
authors.initials: C
authors.lastname: Vicente
authors.forename: M A
authors.initials: MA
authors.lastname: Teixeira
authors.forename: O
authors.initials: O
authors.lastname: Martins
authors.forename: J M
authors.initials: JM
authors.lastname: Cabeda
authors.forename: R
authors.initials: R
authors.lastname: Lacerda
authors.forename: C
authors.initials: C
authors.lastname: Goncalves
authors.forename: J
authors.initials: J
authors.lastname: Fraga
authors.forename: G
authors.initials: G
authors.lastname: Macedo
authors.forename: B M
authors.initials: BM
authors.lastname: Silva
authors.forename: H
authors.initials: H
authors.lastname: Alves
authors.forename: B
authors.initials: B
authors.lastname: Justiça
authors.forename: M
authors.initials: M
authors.lastname: de Sousa
mesh_terms: D000328:Adult; D000367:Age Factors; D000368:Aged; D016516:CD4-CD8 Ratio; D015897:Comorbidity; D005260:Female; D006680:HLA Antigens; D006432:Hemochromatosis / Q000453:epidemiology / Q000276:immunology* / Q000378:metabolism; D006801:Humans; D007501:Iron / Q000378:metabolism; D008103:Liver Cirrhosis / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D010641:Phenotype; D012737:Sex Factors
chemical_list: D006680:HLA Antigens; D007501:Iron