Document 0818
 DOCN  M9460818
 TI    Epstein-Barr virus-related lymphomagenesis in a child with
       Wiskott-Aldrich syndrome.
 DT    9404
 AU    Gulley ML; Chen CL; Raab-Traub N; Department of Pathology, University of
       North Carolina, Chapel; Hill.
 SO    Hematol Oncol. 1993 May-Jun;11(3):139-45. Unique Identifier : AIDSLINE
       MED/94156331
 AB    Epstein-Barr virus (EBV) DNA was detected in immunoblastic lymphoma
       arising in a child with the primary immunodeficiency, Wiskott-Aldrich
       syndrome. Southern blot analysis of the structure of the EBV genome
       revealed that the lymphoma was monoclonal and contained episomal EBV
       DNA. The EBV latent genes, latent membrane protein 1 (LMP1) and the EBV
       nuclear antigen 2 (EBNA2), were detected by immunohistochemistry in the
       Wiskott-Aldrich lymphoma but not in an EBV-positive Burkitt's lymphoma,
       implying that host immune factors could influence EBV gene expression.
       Hybridization in situ demonstrated expression of EBV-encoded RNA (EBER),
       the cellular c-fgr protooncogene, and CD23 B-cell activation transcripts
       in the Wiskott-Aldrich lymphoma whereas EBER and c-fgr but not CD23 were
       expressed in the Burkitt's lymphoma. These data suggest that in primary
       immunodeficiency-related lymphoma, LMP1 and EBNA2 are expressed and that
       this expression correlates with expression of CD23. This supports
       previous in vitro studies showing that CD23 is specifically induced by
       LMP1 or EBNA2 genes. In contrast, expression of c-fgr may be independent
       of expression of these EBV latent genes.
 DE    Adolescence  Antigens, Viral/ANALYSIS  Colonic Neoplasms/*ETIOLOGY
       DNA-Binding Proteins/ANALYSIS  DNA, Viral/ANALYSIS  Herpesvirus 4,
       Human/GENETICS/*PATHOGENICITY  Human  Lymphoma/*ETIOLOGY  Male
       Proto-Oncogene Proteins/GENETICS  Proto-Oncogenes  Receptors,
       IgE/ANALYSIS  RNA, Viral/ANALYSIS  Support, Non-U.S. Gov't  Support,
       U.S. Gov't, Non-P.H.S.  Support, U.S. Gov't, P.H.S.  Wiskott-Aldrich
       Syndrome/*COMPLICATIONS  JOURNAL ARTICLE

       SOURCE: National Library of Medicine.  NOTICE: This material may be
       protected by Copyright Law (Title 17, U.S.Code).