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Their mutational landscape and the impact of these mutations on survival outcomes remain underexplored. In this study, we characterized nTCL features in a Slovenian cohort, comprising 91 nTFHL, 9 peripheral TCL, not otherwise specified (PTCL-NOS), and 8 composite lymphomas [co-occurrence of nTFHL, angioimmunoblastic type (nTFHL-AI) with monoclonal B-cell proliferation], with a median follow up of 23 months. Using immunohistochemistry, clonality testing, and high-throughput sequencing with a lymphoma panel targeting 172 genes, we analyzed their mutational landscape and examined the mutational impact on survival outcomes. The mutational analysis uncovered\n                    <jats:italic>TET2<\/jats:italic>\n                    (43%),\n                    <jats:italic>RHOA<\/jats:italic>\n                    (26%),\n                    <jats:italic>IDH2<\/jats:italic>\n                    (9%),\n                    <jats:italic>PLCG1<\/jats:italic>\n                    (8%), and\n                    <jats:italic>DNMT3A<\/jats:italic>\n                    (6%) as the most commonly mutated genes.\n                    <jats:italic>RHOA<\/jats:italic>\n                    mutations correlated with\n                    <jats:italic>TET2<\/jats:italic>\n                    ,\n                    <jats:italic>IDH2<\/jats:italic>\n                    , and\n                    <jats:italic>DNMT3A<\/jats:italic>\n                    mutations, and CD10 expression was linked to\n                    <jats:italic>TET2<\/jats:italic>\n                    mutations. Multiple mutations, high International Prognostic Index, progressive disease after first-line treatment, and the absence of autologous stem cell transplantation (ASCT) were independent predictors for shorter survival. To date, this study is one of the largest nTCL series, confirming that nTFHLs outnumber PTCL-NOS (92% vs. 8%). Our findings underscore the complex role of genetic factors in nTCL\u2019s clinical behaviour and emphasize the importance of ASCT. We also highlight the need for prospective clinical trials, which explore tailored therapeutic interventions, such as hypomethylating agents or IDH inhibitors, for improving survival in specific genetic contexts.\n                  <\/jats:p>","DOI":"10.1007\/s00277-025-06709-z","type":"journal-article","created":{"date-parts":[[2025,11,13]],"date-time":"2025-11-13T01:51:34Z","timestamp":1762998694000},"page":"6237-6249","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Insights into Nodal T-Follicular Helper Cell lymphomas and Peripheral T-Cell Lymphomas, Not Otherwise Specified, in Slovenian Patients: Mutational Landscape, Clinicopathological Characteristics, and Outcomes"],"prefix":"10.1007","volume":"104","author":[{"ORCID":"https:\/\/orcid.org\/0009-0005-8230-8707","authenticated-orcid":false,"given":"Eva","family":"Erzar","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5271-537X","authenticated-orcid":false,"given":"Vanesa","family":"Sindi-Ivanova","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0008-5250-3159","authenticated-orcid":false,"given":"Stefan","family":"Dirnhofer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lu\u010dka","family":"Bolte\u017ear","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5235-2054","authenticated-orcid":false,"given":"Janja","family":"Ocvirk","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0605-6596","authenticated-orcid":false,"given":"Veronika","family":"Kloboves Prevodnik","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1100-3819","authenticated-orcid":false,"given":"Alexandar","family":"Tzankov","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8332-3627","authenticated-orcid":false,"given":"Gorana","family":"Ga\u0161ljevi\u0107","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2025,11,13]]},"reference":[{"issue":"1","key":"6709_CR1","doi-asserted-by":"publisher","first-page":"265","DOI":"10.1007\/s00428-022-03412-6","volume":"482","author":"AL Feldman","year":"2023","unstructured":"Feldman AL, Laurent C, Narbaitz M et al (2023) Classification and diagnostic evaluation of nodal T- and NK-cell lymphomas. 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V.S.I. supported the methodology and formal analysis. V.S.I., S.D., L.B., J.O., V.K.P, A.T., and G.G. contributed to the review and editing of the manuscript. L.B. also assisted with editing the clinical data. S.D., A.T., and G.G. re-evaluated the initial diagnoses. A.T. and G.G. provided supervision and conceptualization. All authors read and approved the final article.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Contributions"}},{"value":"Correspondence to Alexandar Tzankov and Gorana Ga\u0161ljevi\u0107.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Corresponding authors"}},{"value":"According to the committee\u2019s recommendation, informed consent was obtained from living patients involved in the study.","order":5,"name":"Ethics","group":{"name":"EthicsHeading","label":"Patient consent"}},{"value":"The authors declare no competing interests.","order":6,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}]}}