Epstein-Barr virus-associated persistent polyclonal B-cell lymphocytosis with a distinct 69-base pair deletion in the LMP1 oncogene.
Epstein-Barr virus (EBV) genomes have been detected in peripheral blood lymphocytes (PBL) of patients with persistent polyclonal B-cell lymphocytosis (PPBL). This is consistent with the hypothesis that latent EBV infection is involved in the pathogenesis of this disorder. Two EBV-encoded proteins expressed in viral latency are the latent membrane proteins 1 and 2A (LMP1 and LMP2A). We have studied the LMP1 oncogene and the LMP2A gene in a female patient with PPBL and her five siblings. A cell line derived from peripheral blood lymphocytes (PBL) of the patient was also analyzed. A distinct 69-base pair deletion was identified within the carboxy terminal NF-kappa B activation domain of the LMP1 oncogene in PBL of the patient and in the cell line, whereas none of the siblings harbored this deletion. The tyrosine-signaling motif and the HLA A2.1 epitope of the LMP2A gene were wild type in the patient and all siblings. The presence of a 69-base pair deletion variant of the LMP1 oncogene within the lymphocytes of a PPBL patient but absence of this deletion variant in the unaffected siblings suggests a direct implication of altered LMP1 oncoprotein-dependent function in the pathogenesis of PPBL.
Larcher
C
C
Institute for Hygiene, University of Innsbruck, Austria.
McQuain
C
C
Berger
C
C
Mitterer
M
M
Quesenberry
P J
PJ
Huemer
H P
HP
Knecht
H
H
D000595:Amino Acid Sequence; D001402:B-Lymphocytes / Q000821:virology*; D001482:Base Composition; D002460:Cell Line / Q000821:virology; D000939:Epitopes / Q000235:genetics; D005260:Female; D017353:Gene Deletion; D015234:HLA-A Antigens / Q000276:immunology; D004854:Herpesvirus 4, Human / Q000737:chemistry / Q000302:isolation & purification*; D006801:Humans; D008218:Lymphocytosis / Q000821:virology*; D008297:Male; D008875:Middle Aged; D008969:Molecular Sequence Data; D017422:Sequence Analysis, DNA; D014763:Viral Matrix Proteins / Q000235:genetics*
C037662:EBV-associated membrane antigen, Epstein-Barr virus; D000939:Epitopes; D015234:HLA-A Antigens; D014763:Viral Matrix Proteins