Nasal T-cell lymphoma: a clinicopathologic entity associated with peculiar phenotype and with Epstein-Barr virus.
Recent evidence has shown that most nasal lymphomas (NL) are associated with a T-cell phenotype and are thus called nasal T-cell lymphomas (NTCL), but little information is available about the T-cell receptor (TCR) expression. The presence of Epstein-Barr virus (EBV) genome has been recently reported in NTCL in Oriental populations in which NL and EBV-associated tumors are more common and in occasional Occidental cases. This prompted us to investigate lymphoma biopsies from 7 non-Oriental patients with NTCL for the expression of natural killer (NK) and T-cell antigens, including TCR proteins, for the presence of EBV-encoded latent membrane protein (LMP) using immunohistochemistry and for the presence of EBV DNA and Epstein-Barr early region (EBER) RNA using in situ hybridization (ISH). Six cases displayed a CD3-, TCR alpha beta-, TCR gamma delta-, CD2+, CD7+, CD5-, CD4-, CD8-, CD56+ phenotype, suggesting that these tumors may be peripheral T-cell lymphomas (PTCL) with extensive loss of T-cell antigens and expression of the NK-cell (CD56) antigen or, alternatively, NK-cell neoplasias. The remaining case was a gamma delta PTCL, as shown by the CD3+, TCR gamma delta+ phenotype and the biallelic gamma and delta TCR gene rearrangements. Using ISH, EBER RNA transcripts were detected in tumor cells in all cases and EBV DNA was shown in the 6 tested cases. In all cases, tumor cells expressed LMP. These findings support the concept that NTCL constitute a distinct group of lymphomas that, in addition to their peculiar clinical features, exhibit an unusual TCR "silent" CD56+ or TCR gamma delta+ phenotype and harbor the EBV. In view of the LMP transforming potential, these data suggest that EBV may play a role in the pathogenesis of NTCL.
Kanavaros
P
P
Département de Pathologie, Hôpital Henri Mondor, Créteil, France.
Lescs
M C
MC
Brière
J
J
Divine
M
M
Galateau
F
F
Joab
I
I
Bosq
J
J
Farcet
J P
JP
Reyes
F
F
Gaulard
P
P
D000293:Adolescent; D000328:Adult; D015703:Antigens, CD / Q000032:analysis*; D004279:DNA, Viral / Q000032:analysis; D005260:Female; D004854:Herpesvirus 4, Human / Q000235:genetics / Q000302:isolation & purification*; D006801:Humans; D007150:Immunohistochemistry; D017403:In Situ Hybridization; D016403:Lymphoma, Large B-Cell, Diffuse / Q000473:pathology; D016399:Lymphoma, T-Cell / Q000276:immunology / Q000382:microbiology / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009669:Nose Neoplasms / Q000276:immunology / Q000382:microbiology / Q000473:pathology*; D012367:RNA, Viral / Q000032:analysis; D011948:Receptors, Antigen, T-Cell / Q000032:analysis*; D012878:Skin Neoplasms / Q000276:immunology / Q000382:microbiology / Q000473:pathology*; D014158:Transcription, Genetic
D015703:Antigens, CD; D004279:DNA, Viral; D012367:RNA, Viral; D011948:Receptors, Antigen, T-Cell