{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,14]],"date-time":"2026-10-14T12:17:22Z","timestamp":1791980242115,"version":"4.4.0"},"reference-count":57,"publisher":"Springer Science and Business Media LLC","issue":"3","license":[{"start":{"date-parts":[[2023,8,30]],"date-time":"2023-08-30T00:00:00Z","timestamp":1693353600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2023,8,30]],"date-time":"2023-08-30T00:00:00Z","timestamp":1693353600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Virchows Arch"],"published-print":{"date-parts":[[2023,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Cytotoxic peripheral T-cell lymphomas and EBV-positive T\/NK-cell lymphoproliferative diseases were discussed at the 2022 European Association for Haematopathology\/Society for Hematopathology lymphoma workshop held in Florence, Italy. This session focused on (i) primary nodal EBV-positive T and NK-cell lymphomas (primary nodal-EBV-TNKL), (ii) extranodal EBV-positive T\/NK lymphoproliferative diseases (LPD) in children and adults, (iii) cytotoxic peripheral T-cell lymphomas, NOS (cPTCL-NOS), EBV-negative, and (iv) miscellaneous cases. Primary nodal-EBV-TNKL is a newly recognized entity which is rare, aggressive, and associated with underlying immune deficiency\/immune dysregulation. All cases presented with lymphadenopathy but some demonstrated involvement of tonsil\/Waldeyer\u2019s ring and extranodal sites. The majority of tumors are of T-cell lineage, and the most frequent mutations involve the epigenetic modifier genes, such as <jats:italic>TET2<\/jats:italic> and <jats:italic>DNMT3A<\/jats:italic>, and JAK-STAT genes. A spectrum of EBV-positive T\/NK LPD involving extranodal sites were discussed and highlight the diagnostic challenge with primary nodal-EBV-TNKL when these extranodal EBV-positive T\/NK LPD cases demonstrate predominant nodal disease either at presentation or during disease progression from chronic active EBV disease. The majority of cPTCL-NOS demonstrated the TBX21 phenotype. Some cases had a background of immunosuppression or immune dysregulation. Interestingly, an unexpected association of cPTCL-NOS, EBV-positive and negative, with TFH lymphomas\/LPDs was observed in the workshop cases. Similar to a published literature, the genetic landscape of cPTCL-NOS from the workshop showed frequent mutations in epigenetic modifiers, including <jats:italic>TET2<\/jats:italic> and <jats:italic>DNMT3A<\/jats:italic>, suggesting a role of clonal hematopoiesis in the disease pathogenesis.<\/jats:p>","DOI":"10.1007\/s00428-023-03616-4","type":"journal-article","created":{"date-parts":[[2023,8,30]],"date-time":"2023-08-30T06:02:07Z","timestamp":1693375327000},"page":"333-348","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":24,"title":["Cytotoxic peripheral T-cell lymphomas and EBV-positive T\/NK-cell lymphoproliferative diseases: emerging concepts, recent advances, and the putative role of clonal hematopoiesis. A report of the 2022 EA4HP\/SH lymphoma workshop"],"prefix":"10.1007","volume":"483","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4360-8388","authenticated-orcid":false,"given":"Fina","family":"Climent","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alina","family":"Nicolae","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3994-516X","authenticated-orcid":false,"given":"Laurence","family":"de Leval","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefan","family":"Dirnhofer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7457-300X","authenticated-orcid":false,"given":"Lorenzo","family":"Leoncini","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9431-2889","authenticated-orcid":false,"given":"Sarah L.","family":"Ondrejka","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lorinda","family":"Soma","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0240-387X","authenticated-orcid":false,"given":"Andrew","family":"Wotherspoon","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6203-7966","authenticated-orcid":false,"given":"Alberto","family":"Zamo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7156-5365","authenticated-orcid":false,"given":"Leticia","family":"Quintanilla-Martinez","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6051-6410","authenticated-orcid":false,"given":"Siok-Bian","family":"Ng","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2023,8,30]]},"reference":[{"key":"3616_CR1","doi-asserted-by":"crossref","first-page":"1124","DOI":"10.3389\/fimmu.2017.01124","volume":"8","author":"S Paul","year":"2017","unstructured":"Paul S, Lal G (2017) The molecular mechanism of natural killer cells function and its importance in cancer immunotherapy. 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