X-linked lymphoproliferative disease (XLP) as a model of Epstein-Barr virus-induced immunopathology

Purtilo, David T.

doi:10.1007/bf00201468

Cited by 26 publications

(10 citation statements)

References 60 publications

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“…The X-linked lymphoproliferative disorder (XLPD) is characterized by uncontrolled EBV infections, leading to severe or fulminant infectious mononucleosis, acquired agammaglobulinemia and malignant lymphoma [1]. The inability of the immune system to eliminate EBV-infected B cells is due to defects of helper T cell, cytotoxic T cell and NK cell responses [2][3][4].…”

Section: Introductionmentioning

confidence: 99%

Patients with X-linked lymphoproliferative disease have a defect in 2B4 receptor-mediated NK cell cytotoxicity

Nakajima

Cella²,

Bouchon³

et al. 2000

Eur. J. Immunol.

155

102

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A significant breakthrough in the nitride chemistry of main group elements has been achieved: Surprisingly Sn3N4, for which there had been no proof of existence until now, as well as the isotypic high‐pressure polymorphs γ‐Si3N4 and γ‐Ge3N4 have been synthesized. All three compounds crystallize in a spinel structure type (see picture) that previously has not been found for nitrides, and may have a high relevance for practical applications in the context of ultrahard materials.

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Section: Introductionmentioning

confidence: 99%

Patients with X-linked lymphoproliferative disease have a defect in 2B4 receptor-mediated NK cell cytotoxicity

Nakajima

Cella²,

Bouchon³

et al. 2000

Eur. J. Immunol.

155

102

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“…It is 1 of the 6 human X-linked immunodeficiencies, but its close relationship to Epstein-Barr virus (EBV) is exceptional. Some patients develop meningitis or pneumonitis of bacterial or viral origin (Purtilo, 1980(Purtilo, , 1991. B cells of XLP patients fail to produce normal IgM levels in vitro and do not switch to IgG after challenge with bacteriophage ⌽ X174 (Purtilo et al, 1989).…”

mentioning

confidence: 99%

SH2D1A and slam protein expression in human lymphocytes and derived cell lines

et al. 2000

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The gene defect responsible for the X-linked lymphoproliferative disease (XLP) is associated with an impaired control of Epstein-Barr virus (EBV) infection. The gene has been recently identified and the encoded protein (designated SH2D1A, DSHP or SAP) was characterized. It is a 128 amino acid (aa) protein, containing a single Src homology 2 (SH2) domain. It interacts with signaling lymphocytic activation molecule (SLAM) expressed on the surface of activated T and B cells. We show that activated T, but not activated B, cells express the SH2D1A protein. NK cells express the protein as well. Tumor lines originating from B, T or NK cells exhibited similar SH2D1A protein expression as the corresponding normal cells, with some notable exceptions. EBV-carrying, tumor phenotype representative (type I), but not EBV-carrying lymphoblastoid cell line (LCL)-like (type III) or EBV-negative Burkitt lymphoma (BL) lines expressed SH2D1A. The phenotypic switch from type I to type III in the EBV-carrying BL line Mutu was associated with a down-regulation of SH2D1A and up-regulation of SLAM. In contrast to normal ex vivo and long-term activated NK cells, 2 of 3 NK leukemia lines expressed SLAM. All 3 lines expressed SH2D1A, like their normal counterparts.

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“…Fatal infectious mononucleosis occurs as an X–linked lymphoproliferative syndrome (XLP) in males [11, 14, 15]. In addition, Sakamoto et al [16]and others described a sporadic form of fatal mononucleosis in previously healthy males and females.…”

Section: Discussionmentioning

confidence: 99%

Atypical CD3+ CD4<sup>low</sup> Cell Population in a Boy with Fatal EBV–Infection

Gruber¹,

Krauss‐Etschmann²,

Jäger

et al. 1999

Int Arch Allergy Immunol

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A previously healthy 10–year–old Greek boy born to nonconsanguineous healthy parents developed progressive liver disease after acute infectious mononucleosis. EBV–induced autoimmune hepatitis was suspected and treatment was started with high–dose prednisolone, acyclovir and intravenous immunoglobulins. Despite therapy, his liver function continuously deteriorated and the child died 9 months later in profound immune deficiency from candida septicemia. Flow cytometric analysis of his lymphocytes revealed a major subpopulation of atypical cells (20.3%) which were CD3+, fitted into the lymphocyte gate but showed a very low level of CD4 expression, comparable to that of monocytes. After short–time cell culture, the cells became adherent and developed granules and dendrites. We conclude that these cells may represent strongly activated CD4+ T lymphocytes with downregulated CD4 expression or a subtype of dendritic cells.

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X-linked lymphoproliferative disease (XLP) as a model of Epstein-Barr virus-induced immunopathology

Cited by 26 publications

References 60 publications

Patients with X-linked lymphoproliferative disease have a defect in 2B4 receptor-mediated NK cell cytotoxicity

Patients with X-linked lymphoproliferative disease have a defect in 2B4 receptor-mediated NK cell cytotoxicity

SH2D1A and slam protein expression in human lymphocytes and derived cell lines

Atypical CD3+ CD4<sup>low</sup> Cell Population in a Boy with Fatal EBV–Infection

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