title: Epstein-Barr virus-positive nodal T/NK-cell lymphoma: an analysis of 15 cases with distinct clinicopathological features.
abstract: Nodal peripheral T-cell lymphoma, not otherwise specified, is a heterogeneous entity with variable biologic behavior. We analyze the clinicopathological features of 15 patients with Epstein-Barr virus-positive (EBV+) nodal T/NK-cell lymphoma, including 9 males and 6 females, with a median age of 64 years. All patients presented with multiple lymphadenopathy with common B symptoms (80%, 12/15) at an advanced Ann Arbor stage (III, IV) (87%, 13/15). The International Prognostic Index was high or high/intermediate in 87% (13/15) of patients, and the prognostic index for peripheral T-cell lymphoma was group 3 or 4 in 73% (11/15). Spleen and liver involvement was observed in 73% (11/15) and 60% (9/15) of patients, respectively. In contrast, extranodal involvement was infrequent, with no more than 1 site in 71% (10/15) of patients. Moreover, none had nasal lesions, and only 1 had mucocutaneous involvement. The cell lineage of EBV+ tumor cells was determined to be T cell in all except 1 patient, who was NK-cell lineage. Cytotoxic molecules were expressed in all cases, and 64% (9/14) of patients expressed the αβT-cell receptor. Moreover, most patients (67%, 10/15) showed CD8 positivity, with 2 of them being CD4CD8 double positive; the others were CD4 positive (n = 2) or CD4CD8 double negative (n = 3). The clinical course was very aggressive, with a median survival time of 3.5 months, and 10 patients died within 6 months of diagnosis. Taken together, our data demonstrate that EBV+ nodal T/NK-cell lymphoma is a distinct clinicopathological entity characterized by cytotoxic molecule expression, a frequent CD8-positive αβT-cell lineage, and a very aggressive clinical behavior.
authors.affiliation: Department of Pathology, Seoul National University Hospital, Seoul 110-744, Republic of Korea.
authors.forename: Yoon Kyung
authors.initials: YK
authors.lastname: Jeon
authors.affiliation: Department of Pathology, Chonnam National University Medical School, Gwangju 500-872, Republic of Korea.
authors.forename: Jo-Heon
authors.initials: JH
authors.lastname: Kim
authors.affiliation: Department of Pathology, Kyung Hee University School of Medicine, Seoul 130-872, Republic of Korea.
authors.forename: Ji-Youn
authors.initials: JY
authors.lastname: Sung
authors.affiliation: Department of Pathology, Ajou University School of Medicine, Suwon, Gyeonggi-do 443-380, Republic of Korea. Electronic address: hanpathol@naver.com.
authors.forename: Jae Ho
authors.initials: JH
authors.lastname: Han
authors.affiliation: Department of Pathology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul 135-710, Republic of Korea.
authors.forename: Young-Hyeh
authors.initials: YH
authors.lastname: Ko
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D014408:Biomarkers, Tumor / Q000032:analysis; D001706:Biopsy; D015496:CD4-Positive T-Lymphocytes / Q000276:immunology* / Q000821:virology; D018414:CD8-Positive T-Lymphocytes / Q000276:immunology* / Q000821:virology; D019070:Cell Lineage; D020031:Epstein-Barr Virus Infections / Q000175:diagnosis* / Q000276:immunology / Q000401:mortality / Q000473:pathology / Q000628:therapy / Q000821:virology; D005260:Female; D004854:Herpesvirus 4, Human / Q000235:genetics / Q000302:isolation & purification*; D006801:Humans; D007150:Immunohistochemistry; D017403:In Situ Hybridization; D053208:Kaplan-Meier Estimate; D007694:Killer Cells, Natural / Q000276:immunology* / Q000821:virology; D054391:Lymphoma, Extranodal NK-T-Cell / Q000175:diagnosis* / Q000276:immunology / Q000401:mortality / Q000473:pathology / Q000628:therapy / Q000821:virology; D016411:Lymphoma, T-Cell, Peripheral / Q000175:diagnosis* / Q000276:immunology / Q000401:mortality / Q000473:pathology / Q000628:therapy / Q000821:virology; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D012367:RNA, Viral / Q000235:genetics; D016693:Receptors, Antigen, T-Cell, alpha-beta / Q000032:analysis; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D014408:Biomarkers, Tumor; D012367:RNA, Viral; D016693:Receptors, Antigen, T-Cell, alpha-beta
keywords: Cytotoxic molecule; EBV positive nodal T/NK-cell lymphoma; Epstein-Barr virus; Extranodal NK/T-cell lymphoma; Not otherwise specified; Peripheral T-cell lymphoma