Important note: Information in this article was accurate in 1992. The state of the art may have changed since the publication date.
EBV-ASSOCIATED B CELL LYMPHOPROLIFERATIVE DISORDERS IN IMMUNODEFICIENCY: THERAPEUTIC STRATEGIES (MEETING ABSTRACT)
Causes, Cures and Consequences of Lymphoproliferative Diseases--A Symposium to Honor George and Eva Klein. May 9-11, 1991, Omaha, NE, 1991.. Unique Identifier : AIDSLINE ICDB/92679942 Shapiro RS; Univ. of Minnesota, Minneapolis, Minnesota, 55455
Abstract:
Epstein-Barr virus (EBV)-associated B cell lymphoproliferative disorder (BLPD) is a recognized complication of primary (congenital) immunodeficiency as well as secondary (acquired or iatrogenic) immunodeficiency such as after organ transplantation, following bone marrow transplantation, and in patients (pts) with AIDS. Although the nature of the immune defect(s) that predispose to the development of BLPD are unknown, it is postulated that aberrant EBV-specific T-cell responses are involved. It is our hypothesis that unbalanced lymphokine production is a major contributory factor to abnormal B-cell growth in response to EBV, resulting in BLPD. Since interferon alpha (IFN-alpha) and interleukin 4 (IL-4) have been found to be major regulators of normal B-cell proliferation and also regulate the synthesis of immunoglobulin E, we determined serum levels of IFN-alpha, IL-4 and IgE in 6 pts with newly diagnosed BLPD and 10 normal EBV seropositive controls. In comparison with healthy EBV-seropositive individuals, pts with BLPD exhibited (a) significantly lower levels of serum IFN-alpha, (b) significantly higher levels of serum IL-4, and (c) significantly higher levels of serum IgE. These results suggest that imbalance in the proportions of circulating lymphokines favoring B-cell proliferation may be contributing to the development of EBV-associated BLPD. The significance of these findings is exemplified by our recent success in the treatment of BLPD with IFN-alpha and iv immunoglobulin. It is our hope that through better understanding of the pathophysiology of EBV-associated BLPD in immunodeficient individuals, better prophylactic and therapeutic regimens can be developed.
Keywords: Acquired Immunodeficiency Syndrome/COMPLICATIONS B-Lymphocytes/CYTOLOGY Bone Marrow Transplantation/ADVERSE EFFECTS Cell Division *Herpesvirus 4, Human Human IgE/BIOSYNTHESIS Immunologic Deficiency Syndromes/*COMPLICATIONS Interferon-alpha/BLOOD Interleukin-4/BLOOD Lymphokines/METABOLISM Lymphoma, B-Cell/IMMUNOLOGY/*THERAPY T-Lymphocytes/IMMUNOLOGY ABSTRACT
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