{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,28]],"date-time":"2026-08-28T00:08:19Z","timestamp":1787875699303,"version":"build-2784847793"},"reference-count":24,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2023,11,7]],"date-time":"2023-11-07T00:00:00Z","timestamp":1699315200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["pathsocjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["The Journal of Pathology"],"published-print":{"date-parts":[[2024,2]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Primary cutaneous CD4+ small or medium T\u2010cell lymphoproliferative disorder (PCSM\u2010LPD) is a clonal T\u2010cell proliferation disease confined to the skin. PCSM\u2010LPD shares expression of T follicular helper (Tfh) cell markers with various mature T\u2010cell lymphomas. However, the benign presentation of PCSM\u2010LPD contrasts the clinical behavior of other Tfh\u2010lymphomas. The aim of our study was to delineate the molecular similarities and differences between PCSM\u2010LPD and other Tfh\u2010derived lymphomas to explain the clinical behavior and unravel possible pathological mechanisms. We performed targeted next\u2010generation sequencing of 19 genes recurrently mutated in T\u2010cell neoplasms in\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u200917 PCSM\u2010LPD with high and in\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u200921 PCSM\u2010LPD with low tumor cell content. Furthermore, gene expression profiling was used to identify genes potentially expressed in the PD1\u2010positive (PD1+) neoplastic cells. Expression of some of these genes was confirmed\n                    <jats:italic>in situ<\/jats:italic>\n                    using multistain immunofluorescence. We found that PCSM\u2010LPD rarely harbored mutations recurrently detected in other T\u2010cell neoplasms. PCSM\u2010LPD is characterized by the invariable expression of the T\u2010cell\u2010receptor\u2010associated LCK protein. CD70 and its ligand CD27 are co\u2010expressed on PD1+ PCSM\u2010LPD cells, suggestive of autoactivation of the CD70 pathway. In conclusion, PCSM\u2010LPD differs from disseminated lymphomas of Tfh origin by their mutation profile. Activation of CD70 signaling also found in cutaneous T\u2010cell lymphoma represents a potential driver of neoplastic proliferation of this benign neoplasia of Tfh. \u00a9 2023 The Authors.\n                    <jats:italic>The Journal of Pathology<\/jats:italic>\n                    published by John Wiley &amp; Sons Ltd on behalf of The Pathological Society of Great Britain and Ireland.\n                  <\/jats:p>","DOI":"10.1002\/path.6222","type":"journal-article","created":{"date-parts":[[2023,11,7]],"date-time":"2023-11-07T05:53:54Z","timestamp":1699336434000},"page":"189-197","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["<scp>CD27<\/scp>\n                    \/\n                    <scp>CD70<\/scp>\n                    pathway activation in primary cutaneous\n                    <scp>CD4<\/scp>\n                    + small\/medium T\u2010cell lymphoproliferative disorder"],"prefix":"10.1002","volume":"262","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9543-4084","authenticated-orcid":false,"given":"Julia","family":"Richter","sequence":"first","affiliation":[{"name":"Department of Pathology, Hematopathology Section University Hospital Schleswig\u2010Holstein  Kiel Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ilske","family":"Oschlies","sequence":"additional","affiliation":[{"name":"Department of Pathology, Hematopathology Section University Hospital Schleswig\u2010Holstein  Kiel Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katharina","family":"Kock","sequence":"additional","affiliation":[{"name":"Department of Pathology, Hematopathology Section University Hospital Schleswig\u2010Holstein  Kiel Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"W\u00fcseke","sequence":"additional","affiliation":[{"name":"Department of Pathology, Hematopathology 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