Interferon-gamma in a family with X-linked lymphoproliferative syndrome with acute Epstein-Barr virus infection.
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-gamma), defervescence occurred and circulating EBNA-positive cells markedly decreased. IFN-gamma prior to treatment ranged from 10.8 to 24.5 U/ml in the patient's serum and increased linearly post exogenous IFN-gamma 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-gamma. Defective cytotoxic T cells against EBV-infected cells seem to be responsible for the fulminant infectious mononucleosis in this patient.
Okano
M
M
Department of Pathology and Microbiology, University of Nebraska Medical Center, Omaha 68105-1065.
Thiele
G M
GM
Kobayashi
R H
RH
Davis
J R
JR
Synovec
M S
MS
Grierson
H L
HL
Jaffe
H S
HS
Purtilo
D T
DT
D000208:Acute Disease; D000328:Adult; D000914:Antibodies, Viral / Q000032:analysis; D002648:Child; D002675:Child, Preschool; D005190:Family; D005260:Female; D005334:Fever / Q000628:therapy; D008040:Genetic Linkage; D004854:Herpesvirus 4, Human / Q000276:immunology; D006801:Humans; D007223:Infant; D007244:Infectious Mononucleosis / Q000276:immunology* / Q000628:therapy; D007371:Interferon-gamma / Q000096:biosynthesis / Q000276:immunology* / Q000627:therapeutic use; D007694:Killer Cells, Natural / Q000276:immunology; D008232:Lymphoproliferative Disorders / Q000235:genetics* / Q000276:immunology; D008297:Male; D011994:Recombinant Proteins; D014960:X Chromosome
D000914:Antibodies, Viral; D011994:Recombinant Proteins; D007371:Interferon-gamma