Epstein-Barr virus (EBV)-related lymphoproliferative disorder with subsequent EBV-negative T-cell lymphoma.
A 58-year-old Chinese man presented initially with generalized lymphadenopathy, and lymph-node biopsy showed disturbed architecture with preponderance of large B-blasts mixed with numerous CD8+ T lymphocytes, consistent with an acute Epstein-Barr virus (EBV) infection. Immunohistological and gene rearrangement studies confirmed the absence of clonal T or B cells. Polyclonal EBV with lytic infection was detected by Southern blot hybridization (SoBH). Expression of EBV proteins (EBNA2, LMP and ZEBRA) was detected in a proportion of cells by immunostaining. EBV-lytic proteins EA-D, VCA, MA were also detected in rare scattered cells. Double immunostaining showed that the LMP-positive cells were of B and of T phenotype: 73% CD19+, 26% CD2+, 23% CD3+, 8% CD4+, 17% CD8+. After biopsy, there was spontaneous regression of lymph-node enlargement, but lymphadenopathy recurred 8 months later, and the second lymph-node biopsy showed T-cell lymphoma, confirmed by detection of clonally rearranged T-cell-receptor beta-chain gene. However, EBV genome could not be detected in the second biopsy by SoBH, in situ hybridization for EBV-encoded EBER RNA, and immunostaining for EBNA2, LMP and ZEBRA was also negative. This case is of special interest because an EBV-negative T-cell lymphoma developed shortly after an acute episode of EBV-related lymphoproliferation, even though many EBV-positive T cells were detected during the acute episode. EBV was apparently not a direct cause of the lymphoma, but the close temporal association of the 2 lesions supports the hypothesis that EBV can act as a co-factor in lymphomagenesis.
Tao
Q
Q
Department of Pathology, University of Hong Kong, Queen Mary Hospital.
Srivastava
G
G
Loke
S L
SL
Liang
R H
RH
Liu
Y T
YT
Ho
F C
FC
D015321:Gene Rearrangement; D015333:Gene Rearrangement, beta-Chain T-Cell Antigen Receptor; D005803:Genes, Immunoglobulin; D006566:Herpesviridae Infections / Q000382:microbiology* / Q000473:pathology; D004854:Herpesvirus 4, Human / Q000235:genetics; D006801:Humans; D007150:Immunohistochemistry; D008198:Lymph Nodes / Q000737:chemistry / Q000276:immunology / Q000473:pathology; D016399:Lymphoma, T-Cell / Q000382:microbiology* / Q000473:pathology; D008232:Lymphoproliferative Disorders / Q000382:microbiology* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D012342:RNA, Small Nuclear / Q000032:analysis; D012367:RNA, Viral / Q000032:analysis; D013601:T-Lymphocytes / Q000382:microbiology; D014412:Tumor Virus Infections / Q000382:microbiology* / Q000473:pathology; D014764:Viral Proteins / Q000032:analysis
D012342:RNA, Small Nuclear; D012367:RNA, Viral; D014764:Viral Proteins