title: Absence of complement receptor type 3 and lymphocyte function antigen 1 causing deficient phagocyte and lymphocyte functions.
abstract: We describe a patient with delayed umbilical cord detachment, recurrent bacterial infections, and inability to form pus, despite persistent leucocytosis. Immunofluorescence studies with specific monoclonal antibodies showed a severe deficiency in the expression of alpha-chains of the receptor for the C3bi fragment of C3, complement receptor type 3, and the lymphocyte function antigen 1 molecule, found on neutrophil, monocyte and lymphocyte membranes. These membrane antigen defects were responsible for abnormalities in adhesive cell functions. Polymorphonuclear leucocytes demonstrated a markedly reduced chemiluminescence response as well as an impaired nitroblue tetrazolium test and superoxide generation to a particulate stimulus (zymosan), while the responses to a soluble stimulus (phorbol myristate acetate) were normal. In addition, random migration und chemotactic response to zymosan-activated serum were impaired. The lymphocytes demonstrated abolished natural killer cell cytotoxicity as well as abnormal humoral immunity and a lack of antibody response to pertussis and tetanus antigens.
authors.affiliation: Department of Paediatrics, Division of Immunology, Zürich, Switzerland.
authors.forename: M M
authors.initials: MM
authors.lastname: Manzionna
authors.forename: R A
authors.initials: RA
authors.lastname: Seger
authors.forename: V
authors.initials: V
authors.lastname: Wahn
authors.forename: R M
authors.initials: RM
authors.lastname: Zinkernagel
authors.forename: A
authors.initials: A
authors.lastname: Böcker
authors.forename: W H
authors.initials: WH
authors.lastname: Hitzig
mesh_terms: D000943:Antigens, Differentiation / Q000276:immunology*; D001424:Bacterial Infections / Q000276:immunology; D005260:Female; D006801:Humans; D007154:Immune System Diseases / Q000276:immunology*; D007223:Infant; D007694:Killer Cells, Natural / Q000276:immunology; D016169:Lymphocyte Function-Associated Antigen-1; D009504:Neutrophils / Q000276:immunology; D011951:Receptors, Complement / Q000276:immunology*; D017463:Receptors, Complement 3b; D012008:Recurrence
chemical_list: D000943:Antigens, Differentiation; D016169:Lymphocyte Function-Associated Antigen-1; D011951:Receptors, Complement; D017463:Receptors, Complement 3b