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Angioimmunoblastic T\u2010cell lymphoma (AITL) and some peripheral T\u2010cell lymphoma, not otherwise specified (PTCL\u2010NOS) have similarities to normal CD4<jats:sup>+<\/jats:sup> T\u2010cell subsets in their gene expression profiles. A cell of origin model is, therefore, emerging and is likely to be refined in the future. Follicular helper (Tfh) T cells are now established as the cell of origin of AITL and about 20% of PTCL\u2010NOS. Sequencing studies have identified recurrent genetic alterations in epigenetic modifiers, T\u2010cell receptor signalling pathway intermediates or <jats:italic>RHOA<\/jats:italic>, most commonly a specific mutation leading to RHOA G17V. While PTCL\u2010NOS remains a diagnosis of exclusion, advances in genomics have identified subgroups expressing transcription factors TBX 21 (Th1\u2010like origin) and GATA3 (Th2\u2010like origin). These findings suggest new biomarkers and new therapeutic avenues including the hypomethylating agent azacytidine, or inhibitors of proximal T\u2010cell receptor (TCR) signalling and potentially certain monoclonal antibodies. The advances over the past few years, therefore, prompt stratified medicine approaches to test biologically based treatments and determine the clinical utility of the new disease classifications.<\/jats:p>","DOI":"10.1111\/bjh.16428","type":"journal-article","created":{"date-parts":[[2020,2,17]],"date-time":"2020-02-17T09:45:12Z","timestamp":1581932712000},"page":"54-66","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":47,"title":["The new biology of PTCL\u2010NOS and AITL: current status and future clinical impact"],"prefix":"10.1111","volume":"189","author":[{"given":"Matthew A.","family":"Timmins","sequence":"first","affiliation":[{"name":"Leicester Cancer Research Centre Ernest and Helen Scott Haematology Research Institute University of Leicester Leicester UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8914-0370","authenticated-orcid":false,"given":"Simon 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