Interferon-gamma in a family with X-linked lymphoproliferative syndrome with acute Epstein-Barr virus infection

Motohiko Okano; Geoffrey M. Thiele; Roger H. Kobayashi; Jack R. Davis; Mark S. Synovec; Helen L. Grierson; Howard S. Jaffe; David T. Purtilo

doi:10.1007/BF00917127

Interferon-gamma in a family with X-linked lymphoproliferative syndrome with acute Epstein-Barr virus infection

Motohiko Okano, Geoffrey M. Thiele, Roger H. Kobayashi, Jack R. Davis, Mark S. Synovec, Helen L. Grierson, Howard S. Jaffe, David T. Purtilo

Research output: Contribution to journal › Article › peer-review

30 Scopus citations

Abstract

A 20-month-old male with fulminant infectious mononucleosis and the X-linked lymphoproliferative syndrome (XLP) was studied. Epstein-Barr virus (EBV)-determined nuclear antigen (EBNA) and EBV DNA were detected in various tissues. Despite a combined treatment with acyclovir, immunoglobulin, and methylprednisolone, the patient deteriorated rapidly. Following treatment with recombinant interferon-gamma (IFN-γ), defervescence occurred and circulating EBNA-positive cells markedly decreased. IFN-γ prior to treatment ranged from 10.8 to 24.5 U/ml in the patient's serum and increased linearly post exogenous IFN-γ treatment. His natural killer (NK)-cell activity remained in the normal range throughout his illness but autologous EBV-infected cells were not killed in vitro by his peripheral blood lymphocytes (PBL). These results suggest that patients with the fatal infectious mononucleosis phenotype of XLP may produce endogenous IFN-γ. Defective cytotoxic T cells against EBV-infected cells seem to be responsible for the fulminant infectious mononucleosis in this patient.

Original language	English (US)
Pages (from-to)	48-54
Number of pages	7
Journal	Journal of Clinical Immunology
Volume	9
Issue number	1
DOIs	https://doi.org/10.1007/BF00917127
State	Published - Jan 1989

Keywords

Epstein-Barr virus
X-Linked lymphoproliferative syndrome
cytotoxic T cell
interferon-gamma
natural killer cell

ASJC Scopus subject areas

Immunology and Allergy
Immunology

Access to Document

10.1007/BF00917127

Cite this

@article{d1d9eec294d2402fbd10a5bcd516445f,

title = "Interferon-gamma in a family with X-linked lymphoproliferative syndrome with acute Epstein-Barr virus infection",

abstract = "A 20-month-old male with fulminant infectious mononucleosis and the X-linked lymphoproliferative syndrome (XLP) was studied. Epstein-Barr virus (EBV)-determined nuclear antigen (EBNA) and EBV DNA were detected in various tissues. Despite a combined treatment with acyclovir, immunoglobulin, and methylprednisolone, the patient deteriorated rapidly. Following treatment with recombinant interferon-gamma (IFN-γ), defervescence occurred and circulating EBNA-positive cells markedly decreased. IFN-γ prior to treatment ranged from 10.8 to 24.5 U/ml in the patient's serum and increased linearly post exogenous IFN-γ treatment. His natural killer (NK)-cell activity remained in the normal range throughout his illness but autologous EBV-infected cells were not killed in vitro by his peripheral blood lymphocytes (PBL). These results suggest that patients with the fatal infectious mononucleosis phenotype of XLP may produce endogenous IFN-γ. Defective cytotoxic T cells against EBV-infected cells seem to be responsible for the fulminant infectious mononucleosis in this patient.",

keywords = "Epstein-Barr virus, X-Linked lymphoproliferative syndrome, cytotoxic T cell, interferon-gamma, natural killer cell",

author = "Motohiko Okano and Thiele, {Geoffrey M.} and Kobayashi, {Roger H.} and Davis, {Jack R.} and Synovec, {Mark S.} and Grierson, {Helen L.} and Jaffe, {Howard S.} and Purtilo, {David T.}",

year = "1989",

month = jan,

doi = "10.1007/BF00917127",

language = "English (US)",

volume = "9",

pages = "48--54",

journal = "Journal of Clinical Immunology",

issn = "0271-9142",

publisher = "Springer New York",

number = "1",

}

TY - JOUR

T1 - Interferon-gamma in a family with X-linked lymphoproliferative syndrome with acute Epstein-Barr virus infection

AU - Okano, Motohiko

AU - Thiele, Geoffrey M.

AU - Kobayashi, Roger H.

AU - Davis, Jack R.

AU - Synovec, Mark S.

AU - Grierson, Helen L.

AU - Jaffe, Howard S.

AU - Purtilo, David T.

PY - 1989/1

Y1 - 1989/1

N2 - A 20-month-old male with fulminant infectious mononucleosis and the X-linked lymphoproliferative syndrome (XLP) was studied. Epstein-Barr virus (EBV)-determined nuclear antigen (EBNA) and EBV DNA were detected in various tissues. Despite a combined treatment with acyclovir, immunoglobulin, and methylprednisolone, the patient deteriorated rapidly. Following treatment with recombinant interferon-gamma (IFN-γ), defervescence occurred and circulating EBNA-positive cells markedly decreased. IFN-γ prior to treatment ranged from 10.8 to 24.5 U/ml in the patient's serum and increased linearly post exogenous IFN-γ treatment. His natural killer (NK)-cell activity remained in the normal range throughout his illness but autologous EBV-infected cells were not killed in vitro by his peripheral blood lymphocytes (PBL). These results suggest that patients with the fatal infectious mononucleosis phenotype of XLP may produce endogenous IFN-γ. Defective cytotoxic T cells against EBV-infected cells seem to be responsible for the fulminant infectious mononucleosis in this patient.

AB - A 20-month-old male with fulminant infectious mononucleosis and the X-linked lymphoproliferative syndrome (XLP) was studied. Epstein-Barr virus (EBV)-determined nuclear antigen (EBNA) and EBV DNA were detected in various tissues. Despite a combined treatment with acyclovir, immunoglobulin, and methylprednisolone, the patient deteriorated rapidly. Following treatment with recombinant interferon-gamma (IFN-γ), defervescence occurred and circulating EBNA-positive cells markedly decreased. IFN-γ prior to treatment ranged from 10.8 to 24.5 U/ml in the patient's serum and increased linearly post exogenous IFN-γ treatment. His natural killer (NK)-cell activity remained in the normal range throughout his illness but autologous EBV-infected cells were not killed in vitro by his peripheral blood lymphocytes (PBL). These results suggest that patients with the fatal infectious mononucleosis phenotype of XLP may produce endogenous IFN-γ. Defective cytotoxic T cells against EBV-infected cells seem to be responsible for the fulminant infectious mononucleosis in this patient.

KW - Epstein-Barr virus

KW - X-Linked lymphoproliferative syndrome

KW - cytotoxic T cell

KW - interferon-gamma

KW - natural killer cell

UR - http://www.scopus.com/inward/record.url?scp=0024533509&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0024533509&partnerID=8YFLogxK

U2 - 10.1007/BF00917127

DO - 10.1007/BF00917127

M3 - Article

C2 - 2539385

AN - SCOPUS:0024533509

SN - 0271-9142

VL - 9

SP - 48

EP - 54

JO - Journal of Clinical Immunology

JF - Journal of Clinical Immunology

IS - 1

ER -

Interferon-gamma in a family with X-linked lymphoproliferative syndrome with acute Epstein-Barr virus infection

Abstract

Keywords

ASJC Scopus subject areas

Access to Document

Other files and links

Fingerprint

Cite this