{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T04:13:28Z","timestamp":1790828008964,"version":"4.1.0"},"reference-count":21,"publisher":"S. Karger AG","issue":"6","license":[{"start":{"date-parts":[[2024,3,3]],"date-time":"2024-03-03T00:00:00Z","timestamp":1709424000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/karger.com\/pages\/terms-and-conditions"},{"start":{"date-parts":[[2024,3,3]],"date-time":"2024-03-03T00:00:00Z","timestamp":1709424000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/karger.com\/pages\/terms-and-conditions"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Acta Haematol"],"abstract":"<jats:p>Introduction: Primary nodal Epstein-Barr virus-positive T-cell\/NK-cell lymphoma (PTCL-EBV) is a disease entity newly recognized in the World Health Organization\u2019s classification of hematolymphoid tumors, 5th edition (WHO-HAEMS5) and the International Consensus Classification of Mature Lymphoid Neoplasms (ICC). Previously, it was classified as a subtype within peripheral T-cell lymphoma, not otherwise specified, and was known to have a poor prognosis. However, the clinical features and treatment outcomes are not well known. Methods: This retrospective observational study was conducted on patients diagnosed with PTCL-EBV at Samsung Medical Center through a pathology review from 2000 to 2020. We analyzed the clinical data from 14 patients, immunohistochemistry, and survival outcomes including overall survival (OS) and progression-free survival (PFS) for each treatment regimen. PFS was defined as the time from the start of chemotherapy to the confirmation of disease progression on imaging, and hematopoietic stem-cell transplantation (HSCT) was considered a consolidation treatment. OS was defined as the time from diagnosis to the time of death. Results: 25% (1 out of 4) were beta-F1 positive, and 67% (4 out of 6) were T-cell receptor gamma (TCR\u03b3) positive. T-cell intracellular antigen (TIA-1) and granzyme B exhibited positive results in all cases (3 out of 3), whereas the NK-cell marker CD56 was positive in only 11% of patients (1 out of 9). CD3 was observed in all of the patients (11 out of 11). The CD4 was 43% positive (3 out of 7). The CD8 was investigated in 8 patients, with 37.5% positive (3 out of 8). Hepatosplenomegaly was observed in 55% of patients (6 out of 11), and 70% (7 out of 10) of patients displayed B symptoms at the time of diagnosis. Patients who received CHOP (cyclophosphamide, doxorubicin, vincristine, prednisolone) or CVP (cyclophosphamide, vincristine, prednisolone) treatment had a median PFS of 2.2 months (95% CI: 1.9\u20132.5 months), and patients who received other treatments had a median PFS of 5.1 months (NA). The objective response rate (ORR) for ICE\/dexa (ifosfamide, carboplatin, etoposide, dexamethasone) as the first- or second-line treatment was 100% (3 out of 3). But ORR of CHOP or CVP as the first-line treatment was 33.3% (3 out of 9). The median OS for the group that received HSCT (3 out of 11) after achieving a response was 34.6 months (95% CI: 0\u201374.6 months), and the median OS for the group that did not receive HSCT (8 out of 11) was 5.0 months (95% CI: 2.1\u20137.9 months) (p = 0.04). Conclusions: In conclusion, in the context of PTCL-EBV, despite a limited sample size, the ICE\/Dexa regimen shows potential benefits in terms of ORR and PFS. Furthermore, the application of HSCT following the attainment of a complete response may prove advantageous. <\/jats:p>","DOI":"10.1159\/000537962","type":"journal-article","created":{"date-parts":[[2024,3,4]],"date-time":"2024-03-04T22:00:30Z","timestamp":1709589630000},"page":"625-633","source":"Crossref","is-referenced-by-count":5,"title":["Primary Nodal Epstein-Barr Virus-Positive T-Cell\/NK-Cell Lymphoma: Real-World Experience"],"prefix":"10.1159","volume":"147","author":[{"given":"Dae-Ho","family":"Choi","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sang Eun","family":"Yoon","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Junhun","family":"Cho","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Seok Jin","family":"Kim","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Won Seog","family":"Kim","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"127","published-online":{"date-parts":[[2024,3,3]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","DOI":"10.1038\/s41375-022-01620-2"},{"key":"ref2","doi-asserted-by":"publisher","DOI":"10.1182\/blood.2022015851"},{"key":"ref3","doi-asserted-by":"publisher","DOI":"10.3389\/fonc.2022.863269"},{"key":"ref4","doi-asserted-by":"publisher","DOI":"10.1007\/s12185-014-1520-3"},{"key":"ref5","doi-asserted-by":"publisher","DOI":"10.1016\/j.lanwpc.2021.100126"},{"key":"ref6","doi-asserted-by":"publisher","DOI":"10.1016\/j.humpath.2015.03.002"},{"key":"ref7","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2017.180430"},{"key":"ref8","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2021.280003"},{"key":"ref9","doi-asserted-by":"publisher","DOI":"10.3960\/jslrt.20001"},{"key":"ref10","doi-asserted-by":"publisher","DOI":"10.5402\/2011\/623924"},{"key":"ref11","doi-asserted-by":"publisher","DOI":"10.1093\/annonc\/mdq359"},{"key":"ref12","doi-asserted-by":"publisher","DOI":"10.1159\/000537962"},{"key":"ref13","doi-asserted-by":"publisher","DOI":"10.1023\/a:1011144911781"},{"key":"ref14","doi-asserted-by":"publisher","DOI":"10.1007\/BF02983387"},{"key":"ref15","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2005.05694.x"},{"key":"ref16","doi-asserted-by":"publisher","DOI":"10.1002\/cncr.26629"},{"key":"ref17","doi-asserted-by":"publisher","DOI":"10.1002\/cncr.27752"},{"key":"ref18","doi-asserted-by":"publisher","DOI":"10.1016\/j.prp.2013.04.013"},{"key":"ref19","doi-asserted-by":"publisher","DOI":"10.1007\/s00428-023-03616-4"},{"key":"ref20","doi-asserted-by":"publisher","DOI":"10.3350\/cmh.2021.0277"},{"key":"ref21","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0110650"}],"container-title":["Acta Haematologica"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/karger.com\/aha\/article-pdf\/147\/6\/625\/4306354\/000537962.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/karger.com\/aha\/article-pdf\/147\/6\/625\/4306354\/000537962.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,4,23]],"date-time":"2025-04-23T15:08:15Z","timestamp":1745420895000},"score":1,"resource":{"primary":{"URL":"https:\/\/karger.com\/article\/doi\/10.1159\/000537962"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,3,3]]},"references-count":21,"journal-issue":{"issue":"6","published-online":{"date-parts":[[2024,11,29]]},"published-print":{"date-parts":[[2024,12,2]]}},"URL":"https:\/\/doi.org\/10.1159\/000537962","archive":["Portico"],"relation":{"is-supplemented-by":[{"id-type":"doi","id":"10.6084\/m9.figshare.25331011","asserted-by":"subject"}]},"ISSN":["0001-5792","1421-9662"],"issn-type":[{"value":"0001-5792","type":"print"},{"value":"1421-9662","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,3,3]]}}}