title: NK cell activity and monocyte dysfunctions in a patient with common variable hypogammaglobulinemia.
abstract: A 57-year-old man with a history of recurrent infections from the age of 50 was hospitalized with a diagnosis of common variable hypogammaglobulinemia (CVH). Immunological studies revealed a severe reduction of circulating immunoglobulins of all classes. Immunophenotyping of peripheral blood mononuclear cells (PBMC) with monoclonal antibodies, revealed normal values of total B and T cells with CD4/CD8 ratio sharply reduced (0.35) as compared to normal (1.6) because of an increase of CD8 and a decrease of CD4 cells. The surface expression of IL-2 receptor was normal. Natural cytotoxic and phagocytic system presented several abnormalities: a deep impairment of NK activity was found in spite of a normal number of NK cells, as ascertained by Leu 19 and B73.1 monoclonal antibodies. The defective NK activity was not restored by interferon alfa, but was normalized by recombinant IL-2. Phagocytic function, as defined by zymosan-stimulated O2- production was almost absent. The involvement of natural cytotoxic and phagocytic systems in CVH has been rarely reported; the possible causative role of a chronic viral infection (Epstein-Barr virus?) is discussed, on the base of anamnesis.
authors.affiliation: Istituto Nazionale Tumori e Cattedra di Immunologia, Milano, Italy.
authors.forename: M
authors.initials: M
authors.lastname: Clerici
authors.forename: M L
authors.initials: ML
authors.lastname: Villa
authors.forename: M
authors.initials: M
authors.lastname: Mantovani
authors.forename: C
authors.initials: C
authors.lastname: Rugarli
mesh_terms: D000911:Antibodies, Monoclonal; D001402:B-Lymphocytes / Q000276:immunology; D002461:Cell Line, Transformed; D003602:Cytotoxicity, Immunologic; D004406:Dysgammaglobulinemia / Q000276:immunology*; D006801:Humans; D017098:IgA Deficiency; D017099:IgG Deficiency; D007075:Immunoglobulin M / Q000172:deficiency; D007370:Interferon Type I / Q000494:pharmacology; D007376:Interleukin-2 / Q000494:pharmacology; D007694:Killer Cells, Natural / Q000276:immunology*; D007963:Leukocytes, Mononuclear / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D010587:Phagocytosis; D011994:Recombinant Proteins / Q000494:pharmacology; D050378:T-Lymphocytes, Regulatory / Q000276:immunology
chemical_list: D000911:Antibodies, Monoclonal; D007075:Immunoglobulin M; D007370:Interferon Type I; D007376:Interleukin-2; D011994:Recombinant Proteins