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Biochemical and immunologic abnormalities in peripheral blood T lymphocytes of patients with hemophilia A. 1988

U Dianzani, and A Pileri, and A Bianchi, and A Camponi, and G Tamponi, and M Massaia
Department of Medicine and Experimental Oncology, University of Turin, Italy.

Subset distribution, ecto-5'nucleotidase (5'NT) activity, and the generation of allospecific cytotoxic T lymphocytes (CTL) were investigated in peripheral blood T lymphocytes from 39 hemophilia A patients divided into three groups: group A and group B (HIV-patients with CD4:CD8 ratio less than 1 and greater than 1 respectively), and group C (HIV + patients). 5'NT activity was significantly decreased compared with healthy controls in groups B and C. In group B, this deficiency was attributable to expansion of CD8 + CD11 + suppressor cells. In group C, activated (HLA-DR+) CD8+ cells were also present and contributed to 5'NT deficiency. The suppressor cell expansion seemed to be mainly related to AHF infusions, whereas HLA-DR expression was related to HIV infection. CD11+ and HLA-DR+ cells were also expanded in the CD4+ subpopulation of all three groups, whereas CD4+ CD28+ lymphocytes were significantly decreased in group C only. Lastly, alloreactive cytotoxicity was decreased in group B and was normal in groups A and C.

UI MeSH Term Description Entries
D003602 Cytotoxicity, Immunologic The phenomenon of target cell destruction by immunologically active effector cells. It may be brought about directly by sensitized T-lymphocytes or by lymphoid or myeloid "killer" cells, or it may be mediated by cytotoxic antibody, cytotoxic factor released by lymphoid cells, or complement. Tumoricidal Activity, Immunologic,Immunologic Cytotoxicity,Immunologic Tumoricidal Activities,Immunologic Tumoricidal Activity,Tumoricidal Activities, Immunologic
D006467 Hemophilia A The classic hemophilia resulting from a deficiency of factor VIII. It is an inherited disorder of blood coagulation characterized by a permanent tendency to hemorrhage. Factor VIII Deficiency,Hemophilia,Autosomal Hemophilia A,Classic Hemophilia,Deficiency, Factor VIII,Factor 8 Deficiency, Congenital,Factor VIII Deficiency, Congenital,Haemophilia,Hemophilia A, Congenital,Hemophilia, Classic,As, Autosomal Hemophilia,Autosomal Hemophilia As,Classic Hemophilias,Congenital Hemophilia A,Congenital Hemophilia As,Hemophilia A, Autosomal,Hemophilia As,Hemophilia As, Autosomal,Hemophilia As, Congenital,Hemophilias, Classic
D006679 HIV Seropositivity Development of neutralizing antibodies in individuals who have been exposed to the human immunodeficiency virus (HIV/HTLV-III/LAV). AIDS Seroconversion,AIDS Seropositivity,Anti-HIV Positivity,HIV Antibody Positivity,HIV Seroconversion,HTLV-III Seroconversion,HTLV-III Seropositivity,AIDS Seroconversions,AIDS Seropositivities,Anti HIV Positivity,Anti-HIV Positivities,Antibody Positivities, HIV,Antibody Positivity, HIV,HIV Antibody Positivities,HIV Seroconversions,HIV Seropositivities,HTLV III Seroconversion,HTLV III Seropositivity,HTLV-III Seroconversions,HTLV-III Seropositivities,Positivities, Anti-HIV,Positivities, HIV Antibody,Positivity, Anti-HIV,Positivity, HIV Antibody,Seroconversion, AIDS,Seroconversion, HIV,Seroconversion, HTLV-III,Seroconversions, AIDS,Seroconversions, HIV,Seroconversions, HTLV-III,Seropositivities, AIDS,Seropositivities, HIV,Seropositivities, HTLV-III,Seropositivity, AIDS,Seropositivity, HIV,Seropositivity, HTLV-III
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D013601 T-Lymphocytes Lymphocytes responsible for cell-mediated immunity. Two types have been identified - cytotoxic (T-LYMPHOCYTES, CYTOTOXIC) and helper T-lymphocytes (T-LYMPHOCYTES, HELPER-INDUCER). They are formed when lymphocytes circulate through the THYMUS GLAND and differentiate to thymocytes. When exposed to an antigen, they divide rapidly and produce large numbers of new T cells sensitized to that antigen. T Cell,T Lymphocyte,T-Cells,Thymus-Dependent Lymphocytes,Cell, T,Cells, T,Lymphocyte, T,Lymphocyte, Thymus-Dependent,Lymphocytes, T,Lymphocytes, Thymus-Dependent,T Cells,T Lymphocytes,T-Cell,T-Lymphocyte,Thymus Dependent Lymphocytes,Thymus-Dependent Lymphocyte
D020162 Hyperargininemia A rare autosomal recessive disorder of the urea cycle. It is caused by a deficiency of the hepatic enzyme ARGINASE. Arginine is elevated in the blood and cerebrospinal fluid, and periodic HYPERAMMONEMIA may occur. Disease onset is usually in infancy or early childhood. Clinical manifestations include seizures, microcephaly, progressive mental impairment, hypotonia, ataxia, spastic diplegia, and quadriparesis. (From Hum Genet 1993 Mar;91(1):1-5; Menkes, Textbook of Child Neurology, 5th ed, p51) ARG1 Deficiency,Arginase Deficiency,Arginase Deficiency Disease,Argininemia,Deficiency Disease, Arginase,ARG1 Deficiencies,Arginase Deficiencies,Arginase Deficiency Diseases,Deficiencies, ARG1,Deficiencies, Arginase,Deficiency Diseases, Arginase,Deficiency, ARG1,Deficiency, Arginase,Hyperargininemias

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