Interferon gamma (IFN-gamma) deficiency in generalized Epstein-Barr virus infection with interstitial lymphoid and granulomatous pneumonia, focal cerebral lesions, and genital ulcers: remission following IFN-gamma substitution therapy.

Important note: Information in this article was accurate in 1999. The state of the art may have changed since the publication date.

Clin Infect Dis. 1999 May;28(5):1036-42. Unique Identifier : AIDSLINE MED/99379700
Andersson J; Isberg B; Christensson B; Veress B; Linde A; Bratel T; Department of Infectious Diseases, Karolinska Institutet,; Huddinge University Hospital, Stockholm, Sweden.; jan.andersson@infect.hs.sll.se

Abstract: A 26-year-old previously healthy woman developed granulomatous pneumonitis, encephalitis, and genital ulceration during primary Epstein-Barr virus (EBV) infection. EBV DNA was demonstrated by polymerase chain reaction analysis of serum, lung tissue, and genital ulcer specimens. Serology verified primary EBV infection. The patient lacked lymphocytes cytotoxic to autologous EBV-transformed B lymphocytes. No spontaneous or in vitro EBV-induced interferon gamma (IFN-gamma) production was evident in peripheral blood. The cells had normal IFN-gamma production when stimulated with Staphylococcus aureus exotoxin A. In the bone marrow and peripheral blood, the number of large granular CD56+ lymphocytes (natural killer cells) increased 39%-55%, but no CD4 or CD8 cell lymphocytosis was initially found. A partial clinical response was achieved with treatment with acyclovir, corticosteroids, and intravenous gamma-globulin. Because of persistent granulomatous central nervous system and lung involvement, subcutaneous IFN-gamma therapy was started but was discontinued after 3 months because of development of fever, pancytopenia, and hepatitis. This therapy initiated a complete clinical recovery, which occurred parallel to development of EBV-specific cytotoxic CD8+ T lymphocytes and normalization of natural killer cell lymphocytosis. These findings provide evidence for an EBV-induced lymphoproliferative disorder due to a T lymphocyte dysfunction associated with a selective lack of IFN-gamma synthesis.

Keywords: JOURNAL ARTICLE Adult Case Report CD8-Positive T-Lymphocytes Encephalitis, Viral/ETIOLOGY Epstein-Barr Virus Infections/BLOOD/COMPLICATIONS/IMMUNOLOGY/ *THERAPY Female Genital Diseases, Female/ETIOLOGY Granuloma, Respiratory Tract/ETIOLOGY/PATHOLOGY Human Interferon Type II/ADVERSE EFFECTS/*DEFICIENCY/*THERAPEUTIC USE Lung/IMMUNOLOGY/PATHOLOGY Lung Diseases, Interstitial/ETIOLOGY/PATHOLOGY Lymphoproliferative Disorders/ETIOLOGY/PATHOLOGY Support, Non-U.S. Gov't
991230
A99C1044

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