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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\u2010normal and its EBV\u2010specific antibody\u2010dependent K\u2010cell cytotoxicity (EBV\u2010ADCC) was abnormally low, suggesting that this K\u2010NK cell defect may be primary. The father, who suffered from the syndrome for more than 15 years, lacked (or lost) antibodies to EBV\u2010envelope and infected cell membranes, such as antibody\u2010dependent cellular cytotoxicity (ADCC), neutralizing (NT), and gp 350\/220 antibodies. Slow improvement over a period of years was heralded by rising NK cytotoxicity.<\/jats:p>","DOI":"10.1002\/jmv.1890280211","type":"journal-article","created":{"date-parts":[[2007,3,2]],"date-time":"2007-03-02T16:58:22Z","timestamp":1172854702000},"page":"110-117","source":"Crossref","is-referenced-by-count":50,"title":["Brief report: Killer cell defect and persistent immunological abnormalities in two patients with chronic active epstein\u2010barr virus infection"],"prefix":"10.1002","volume":"28","author":[{"given":"J.","family":"Joncas","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Y.","family":"Monczak","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"F.","family":"Ghibu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C.","family":"Alfieri","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.","family":"Bonin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G.","family":"Ahronheim","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G.","family":"Rivard","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2005,12,11]]},"reference":[{"key":"e_1_2_1_2_1","first-page":"1249","article-title":"Lytic non transforming Epstein\u2010Barr virus (EBV) from a patient with chronic active EBV infection","volume":"131","author":"Alfieri C","year":"1984","journal-title":"Canadian Medical Association Journal"},{"issue":"10","key":"e_1_2_1_3_1","doi-asserted-by":"crossref","first-page":"3306","DOI":"10.1128\/jvi.61.10.3306-3309.1987","article-title":"Biomolecular analysis of a defective nontransforming Epstein\u2010Barr virus (EBV) from a patient with chronic active EBV infection","volume":"61","author":"Alfieri C","year":"1987","journal-title":"Journal of Virology"},{"key":"e_1_2_1_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/0008-8749(86)90001-8"},{"key":"e_1_2_1_5_1","doi-asserted-by":"crossref","first-page":"1513","DOI":"10.1128\/jvi.62.5.1513-1519.1988","article-title":"Recognition of adenovirus E1A gene products on immortalized cell surfaces by cytotoxic T lymphocytes","volume":"62","author":"Bellgrau D","year":"1988","journal-title":"Journal of Virology"},{"key":"e_1_2_1_6_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM198211113072014"},{"key":"e_1_2_1_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(87)90020-1"},{"key":"e_1_2_1_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/ijc.2910130311"},{"key":"e_1_2_1_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(82)80572-6"},{"key":"e_1_2_1_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/0147-9571(79)90008-0"},{"key":"e_1_2_1_11_1","doi-asserted-by":"publisher","DOI":"10.1093\/infdis\/140.2.273"},{"key":"e_1_2_1_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(83)80592-7"},{"key":"e_1_2_1_13_1","unstructured":"JoncasJH(1985):Viral mechanisms of immunosuppression: Cells of the immune system as targets of viral infection and feedback inhibition by suppressor cells and immunoglobulins. 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