title: Brief report: killer cell defect and persistent immunological abnormalities in two patients with chronic active Epstein-Barr virus infection.
abstract: Two members of a family have manifested a syndrome of chronic active Epstein-Barr virus (EBV) infection. A father and his daughter suffered prolonged or recurrent mononucleosis, with splenomegaly, anemia, and intermittent fever; persistent immunological abnormalities included defective natural killer (NK) cytotoxicity, inverted CD4/CD8 ratios, hyper IgG1, high EBV viral capsid antigen (VCA) and early antigen (EA) antibodies, and low or undetectable EBV nuclear antigen (EBNA) antibody titers. The EBV seronegative member of the family was free of these abnormalities. However, NK activity in the seronegative individual was low-normal and its EBV-specific antibody-dependent K-cell cytotoxicity (EBV-ADCC) was abnormally low, suggesting that this K-NK cell defect may be primary. The father, who suffered from the syndrome for more than 15 years, lacked (or lost) antibodies to EBV-envelope and infected cell membranes, such as antibody-dependent cellular cytotoxicity (ADCC), neutralizing (NT), and gp 350/220 antibodies. Slow improvement over a period of years was heralded by rising NK cytotoxicity.
authors.affiliation: Department of Microbiology, Hôpital Sainte-Justine, Montreal, Quebec, Canada.
authors.forename: J
authors.initials: J
authors.lastname: Joncas
authors.forename: Y
authors.initials: Y
authors.lastname: Monczak
authors.forename: F
authors.initials: F
authors.lastname: Ghibu
authors.forename: C
authors.initials: C
authors.lastname: Alfieri
authors.forename: A
authors.initials: A
authors.lastname: Bonin
authors.forename: G
authors.initials: G
authors.lastname: Ahronheim
authors.forename: G
authors.initials: G
authors.lastname: Rivard
mesh_terms: D000293:Adolescent; D000914:Antibodies, Viral / Q000032:analysis; D000920:Antibody-Dependent Cell Cytotoxicity; D000956:Antigens, Viral / Q000032:analysis; D015153:Blotting, Western; D036022:Capsid Proteins; D002648:Child; D002908:Chronic Disease; D019309:Epstein-Barr Virus Nuclear Antigens; D005260:Female; D005455:Fluorescent Antibody Technique; D004854:Herpesvirus 4, Human / Q000276:immunology*; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology; D007244:Infectious Mononucleosis / Q000175:diagnosis / Q000276:immunology* / Q000503:physiopathology; D007370:Interferon Type I / Q000096:biosynthesis; D007371:Interferon-gamma / Q000096:biosynthesis; D007694:Killer Cells, Natural / Q000276:immunology*; D007958:Leukocyte Count; D008297:Male; D008875:Middle Aged; D009500:Neutralization Tests; D012698:Serologic Tests
chemical_list: D000914:Antibodies, Viral; D000956:Antigens, Viral; D036022:Capsid Proteins; D019309:Epstein-Barr Virus Nuclear Antigens; C041788:Epstein-Barr viral capsid antigen; C033305:Epstein-Barr virus early antigen; D007074:Immunoglobulin G; D007370:Interferon Type I; D007371:Interferon-gamma