1986
DOI: 10.1007/bf00918704
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Chediak-Higashi Syndrome: Immunological responses to Epstein-Barr virus studies in gene heterozygotes

Abstract: Immunologic studies were performed in five fathers and nine mothers of patients with Chediak-Higashi Syndrome (CHS). Antibody response to Epstein-Barr virus capsid antigen was higher than in normal controls. Antibodies to diffuse component of the early antigen were not detected and serum antibodies to the restricted component of the early antigen were observed in 64% of the subjects studied. Low natural killer activity and increased proportions of OKT8 positive cells were increased. These data indicate that im… Show more

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Cited by 6 publications
(4 citation statements)
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“…The high levels of HLA-A, -B, and -C molecules and of HLA-E molecules on the surfaces of LCLs are likely to be major factors conferring resistance to NK cell lysis, since mutant LCLs not expressing these surface molecules become sensitive targets (37,60,63). The general resistance of normal LCLs to NK cell-mediated lysis is sometimes overlooked when interpreting the role of NK cells in EBV-associated diseases, such as chronic active EBV infection (21,30), X-linked lymphoproliferative disease (6,37,50), and posttransplant lymphoproliferative disease (56). In these patients, the contribution of NK cell defects to the life-threatening infectious mononucleosis and EBV-induced lymphoma is likely to involve a failure of the NK cells to control lytic EBV infection, with a resultant increase in the viral load and subsequently the frequency of freshly infected cells.…”
Section: Discussionmentioning
confidence: 99%
“…The high levels of HLA-A, -B, and -C molecules and of HLA-E molecules on the surfaces of LCLs are likely to be major factors conferring resistance to NK cell lysis, since mutant LCLs not expressing these surface molecules become sensitive targets (37,60,63). The general resistance of normal LCLs to NK cell-mediated lysis is sometimes overlooked when interpreting the role of NK cells in EBV-associated diseases, such as chronic active EBV infection (21,30), X-linked lymphoproliferative disease (6,37,50), and posttransplant lymphoproliferative disease (56). In these patients, the contribution of NK cell defects to the life-threatening infectious mononucleosis and EBV-induced lymphoma is likely to involve a failure of the NK cells to control lytic EBV infection, with a resultant increase in the viral load and subsequently the frequency of freshly infected cells.…”
Section: Discussionmentioning
confidence: 99%
“…Death may occur at an early age due to infection or a lymphoma-like accelerated phase which may be of Tcell origin (Argyle et aZ., 1982) or rather a lymphohistiocytic proliferation related to EBV infection (Rubin et al, 1985). Other cellular processes that have been shown to be abnormal include absent or decreased natural killer (NK) activity (Haliotis et aZ., 1980;Klein et al, 1980;Merino et al, 1983;Roder and Duwe, 1979), altered responsiveness to EBV infection (Merino et al, 1986), decreased antibody-dependent cell-mediated cytotoxicity (ADCC) (Klein et aL, 1980), and altered neutrophil digestive capabilities (Quie and Cates, 1977). Beige (bg) mice have many of these features, and several groups have proposed that the murine bg mutation is analogous to the mutation in patients with CHS (Brandt et aL, 1975;Lutzner et al, 1966;Windhorst and Padgett, 1973).…”
mentioning
confidence: 99%
“…However, this seems to coincide with the previ ous observation that similar to CHS patients the pa tients' mothers had also reduced NK activities [3] and decreased lysosomal content in polymorphonuclear leukocytes [19],…”
mentioning
confidence: 96%
“…Immunological abnormalities have been demon strated in patients with Chediak-Higashi syndrome (CHS) including a lymphoproliferative disorder [1], changes in subsets of peripheral blood lymphocytes [2] and lowered natural killer cell activity [3,4], In the present study, sera of CHS patients along with those of their mothers were studied for heterophile antibod ies, since such antibodies were shown in a variety of pathologic conditions such as infectious mononucle osis (IM), some malignancies and diseases of un known etiology [see the reviews 5, 6].…”
mentioning
confidence: 99%