{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,2]],"date-time":"2026-10-02T01:12:53Z","timestamp":1790903573732,"version":"4.1.0"},"reference-count":115,"publisher":"MDPI AG","issue":"8","license":[{"start":{"date-parts":[[2022,8,18]],"date-time":"2022-08-18T00:00:00Z","timestamp":1660780800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["20K16204"],"award-info":[{"award-number":["20K16204"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Diagnostics"],"abstract":"<jats:p>Peripheral T-cell lymphomas (PTCLs) are uncommon neoplasms derived from mature T cells or NK cells. PTCLs comprise numerous disease entities, with over 30 distinct entities listed in the latest WHO classification. They predominantly affect adults and elderly people and usually exhibit an aggressive clinical course with poor prognosis. According to their presentation, PTCLs can be divided into nodal, extranodal or cutaneous, and leukemic types. The most frequent primary sites of PTCLs are lymph nodes, with over half of cases showing nodal presentation. Nodal PTCLs include ALK-positive and ALK-negative anaplastic large cell lymphoma; nodal T-cell lymphoma with T follicular helper cell origin; and PTCL, not otherwise specified. Adult T-cell leukemia\/lymphoma also frequently affects lymph nodes. Recent pathological and molecular findings in nodal PTCLs have profoundly advanced the identification of tumor signatures and the refinement of the classification. Therefore, the therapies and pathological diagnosis of nodal PTCLs are continually evolving. This paper aims to provide a summary and update of the pathological and molecular features of nodal PTCLs, which will be helpful for diagnostic practice.<\/jats:p>","DOI":"10.3390\/diagnostics12082001","type":"journal-article","created":{"date-parts":[[2022,8,18]],"date-time":"2022-08-18T21:39:21Z","timestamp":1660858761000},"page":"2001","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":17,"title":["Pathological and Molecular Features of Nodal Peripheral T-Cell Lymphomas"],"prefix":"10.3390","volume":"12","author":[{"given":"Akira","family":"Satou","sequence":"first","affiliation":[{"name":"Department of Surgical Pathology, Aichi Medical University Hospital, Nagakute 480-1195, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Taishi","family":"Takahara","sequence":"additional","affiliation":[{"name":"Department of Surgical Pathology, Aichi Medical University Hospital, Nagakute 480-1195, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4855-4366","authenticated-orcid":false,"given":"Toyonori","family":"Tsuzuki","sequence":"additional","affiliation":[{"name":"Department of Surgical Pathology, Aichi Medical University Hospital, Nagakute 480-1195, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,8,18]]},"reference":[{"key":"ref_1","unstructured":"Swerdlow, S., Campo, E., Harris, N., Jaffe, E., Pileri, S., Stein, H., and Thiele, J. (2017). WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues, Revised, International Agency for Research on Cancer. [4th ed.]."},{"key":"ref_2","first-page":"306","article-title":"Genetic profiling and biomarkers in peripheral T-cell lymphomas: Current role in the diagnostic work-up","volume":"35","author":"Vega","year":"2022","journal-title":"Mod. Pathol. Off. J. USA Can. Acad. Pathol. Inc."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"536","DOI":"10.1111\/bjh.12659","article-title":"Differences in incidence and trends of haematological malignancies in Japan and the United States","volume":"164","author":"Chihara","year":"2014","journal-title":"Br. J. Haematol."},{"key":"ref_4","doi-asserted-by":"crossref","unstructured":"Pileri, S.A., Tabanelli, V., Fiori, S., Calleri, A., Melle, F., Motta, G., Lorenzini, D., Tarella, C., and Derenzini, E. (2021). Peripheral T-Cell Lymphoma, Not Otherwise Specified: Clinical Manifestations, Diagnosis, and Future Treatment. Cancers, 13.","DOI":"10.3390\/cancers13184535"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"3768","DOI":"10.1182\/blood-2004-09-3502","article-title":"WHO-EORTC classification for cutaneous lymphomas","volume":"105","author":"Willemze","year":"2005","journal-title":"Blood"},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"3653","DOI":"10.1182\/blood.V95.12.3653","article-title":"Primary and secondary cutaneous CD30(+) lymphoproliferative disorders: A report from the Dutch Cutaneous Lymphoma Group on the long-term follow-up data of 219 patients and guidelines for diagnosis and treatment","volume":"95","author":"Bekkenk","year":"2000","journal-title":"Blood"},{"key":"ref_7","first-page":"S14","article-title":"Genetics of Breast Implant-Associated Anaplastic Large Cell Lymphoma (BIA-ALCL)","volume":"39","author":"Oishi","year":"2019","journal-title":"Aesthetic Surg. J."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"479","DOI":"10.1093\/ajcp\/aqab012","article-title":"How I Diagnose Anaplastic Large Cell Lymphoma","volume":"155","author":"Amador","year":"2021","journal-title":"Am. J. Clin. Pathol."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1182\/blood-2014-10-567461","article-title":"The biology and management of systemic anaplastic large cell lymphoma","volume":"126","author":"Hapgood","year":"2015","journal-title":"Blood"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"2076","DOI":"10.1182\/blood.V91.6.2076","article-title":"ALK-positive lymphoma: A single disease with a broad spectrum of morphology","volume":"91","author":"Benharroch","year":"1998","journal-title":"Blood"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"4669","DOI":"10.1200\/JCO.2011.36.5411","article-title":"Prognostic impact of morphologic and phenotypic features of childhood ALK-positive anaplastic large-cell lymphoma: Results of the ALCL99 study","volume":"29","author":"Lamant","year":"2011","journal-title":"J. Clin. Oncol. Off. J. Am. Soc. Clin. Oncol."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"545","DOI":"10.1111\/bjh.12777","article-title":"Paediatric anaplastic large cell lymphoma with leukaemic presentation in children: A report of nine French cases","volume":"165","author":"Spiegel","year":"2014","journal-title":"Br. J. Haematol."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"859","DOI":"10.1097\/00000478-199309000-00001","article-title":"A small-cell-predominant variant of primary Ki-1 (CD30)+ T-cell lymphoma","volume":"17","author":"Kinney","year":"1993","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"230","DOI":"10.1111\/pin.12047","article-title":"Lymphohistiocytic and small cell pattern of anaplastic large cell lymphoma, ALK positive, arising in an 86-year-old woman","volume":"63","author":"Satou","year":"2013","journal-title":"Pathol. Int."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"223","DOI":"10.1097\/01.pas.0000179123.66748.c2","article-title":"ALK-positive anaplastic large cell lymphoma mimicking nodular sclerosis Hodgkin\u2019s lymphoma: Report of 10 cases","volume":"30","author":"Vassallo","year":"2006","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"983","DOI":"10.1097\/00000478-199010000-00013","article-title":"Sarcomatoid variant of anaplastic large-cell Ki-1 lymphoma","volume":"14","author":"Chan","year":"1990","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_17","first-page":"219","article-title":"Anaplastic large cell lymphoma: The shifting sands of diagnostic hematopathology","volume":"14","author":"Jaffe","year":"2001","journal-title":"Mod. Pathol. Off. J. USA Can. Acad. Pathol. Inc."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"4005","DOI":"10.1182\/blood.V88.10.4005.bloodjournal88104005","article-title":"Anaplastic large-cell lymphomas of T-cell and null-cell phenotype express cytotoxic molecules","volume":"88","author":"Foss","year":"1996","journal-title":"Blood"},{"key":"ref_19","first-page":"78","article-title":"Approach to nodal-based T-cell lymphomas","volume":"52","year":"2020","journal-title":"Pathology"},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"771","DOI":"10.1111\/bjh.13265","article-title":"Advances in understanding the pathogenesis of systemic anaplastic large cell lymphomas","volume":"168","author":"Boi","year":"2015","journal-title":"Br. J. Haematol."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"915","DOI":"10.1182\/blood-2010-08-303305","article-title":"Discovery of recurrent t(6; 7)(p25.3; q32.3) translocations in ALK-negative anaplastic large cell lymphomas by massively parallel genomic sequencing","volume":"117","author":"Feldman","year":"2011","journal-title":"Blood"},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"1473","DOI":"10.1182\/blood-2014-04-571091","article-title":"ALK-negative anaplastic large cell lymphoma is a genetically heterogeneous disease with widely disparate clinical outcomes","volume":"124","author":"Jaffe","year":"2014","journal-title":"Blood"},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"700","DOI":"10.1182\/blood.2019004164","article-title":"\u201cDouble-hit\u201d of DUSP22 and TP63 rearrangements in anaplastic large cell lymphoma, ALK-negative","volume":"135","author":"Karube","year":"2020","journal-title":"Blood"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"5496","DOI":"10.1182\/blood-2008-01-134270","article-title":"ALK- anaplastic large-cell lymphoma is clinically and immunophenotypically different from both ALK+ ALCL and peripheral T-cell lymphoma, not otherwise specified: Report from the International Peripheral T-Cell Lymphoma Project","volume":"111","author":"Savage","year":"2008","journal-title":"Blood"},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"554","DOI":"10.1182\/blood-2016-12-755496","article-title":"DUSP22 and TP63 rearrangements predict outcome of ALK-negative anaplastic large cell lymphoma: A Danish cohort study","volume":"130","author":"Pedersen","year":"2017","journal-title":"Blood"},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"36","DOI":"10.1097\/PAS.0000000000000500","article-title":"Morphologic Features of ALK-negative Anaplastic Large Cell Lymphomas With DUSP22 Rearrangements","volume":"40","author":"King","year":"2016","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"550","DOI":"10.1097\/PAS.0000000000001614","article-title":"Striking Association of Lymphoid Enhancing Factor (LEF1) Overexpression and DUSP22 Rearrangements in Anaplastic Large Cell Lymphoma","volume":"45","author":"Ravindran","year":"2021","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"19","DOI":"10.1016\/j.humpath.2017.01.003","article-title":"Expression of p63 protein in anaplastic large cell lymphoma: Implications for genetic subtyping","volume":"64","author":"Wang","year":"2017","journal-title":"Hum. Pathol."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"1386","DOI":"10.1182\/blood-2018-03-838524","article-title":"Molecular profiling reveals immunogenic cues in anaplastic large cell lymphomas with DUSP22 rearrangements","volume":"132","author":"Luchtel","year":"2018","journal-title":"Blood"},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"516","DOI":"10.1016\/j.ccell.2015.03.006","article-title":"Convergent mutations and kinase fusions lead to oncogenic STAT3 activation in anaplastic large cell lymphoma","volume":"27","author":"Crescenzo","year":"2015","journal-title":"Cancer Cell"},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"2776","DOI":"10.1182\/blood.2019000626","article-title":"Recurrent MSC (E116K) mutations in ALK-negative anaplastic large cell lymphoma","volume":"133","author":"Luchtel","year":"2019","journal-title":"Blood"},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"e361","DOI":"10.3324\/haematol.2015.126300","article-title":"Angioimmunoblastic T-cell lymphoma is the most common T-cell lymphoma in two distinct French information data sets","volume":"100","author":"Parrens","year":"2015","journal-title":"Haematologica"},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"4124","DOI":"10.1200\/JCO.2008.16.4558","article-title":"International peripheral T-cell and natural killer\/T-cell lymphoma study: Pathology findings and clinical outcomes","volume":"26","author":"Vose","year":"2008","journal-title":"J. Clin. Oncol. Off. J. Am. Soc. Clin. Oncol."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"240","DOI":"10.1200\/JCO.2011.37.3647","article-title":"Clinicopathologic characteristics of angioimmunoblastic T-cell lymphoma: Analysis of the international peripheral T-cell lymphoma project","volume":"31","author":"Federico","year":"2013","journal-title":"J. Clin. Oncol. Off. J. Am. Soc. Clin. Oncol."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"2837","DOI":"10.1182\/blood-2011-08-374371","article-title":"Retrospective analysis of prognostic factors for angioimmunoblastic T-cell lymphoma: A multicenter cooperative study in Japan","volume":"119","author":"Tokunaga","year":"2012","journal-title":"Blood"},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"4463","DOI":"10.1182\/blood-2007-08-105759","article-title":"Clinical, biologic, and pathologic features in 157 patients with angioimmunoblastic T-cell lymphoma treated within the Groupe d\u2019Etude des Lymphomes de l\u2019Adulte (GELA) trials","volume":"111","author":"Mourad","year":"2008","journal-title":"Blood"},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"1077","DOI":"10.1097\/PAS.0b013e31802d68e9","article-title":"Histologic evolution of angioimmunoblastic T-cell lymphoma in consecutive biopsies: Clinical correlation and insights into natural history and disease progression","volume":"31","author":"Attygalle","year":"2007","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"202","DOI":"10.1053\/j.semdp.2011.03.003","article-title":"Follicular helper T cells: Implications in neoplastic hematopathology","volume":"28","author":"Gaulard","year":"2011","journal-title":"Semin. Diagn. Pathol."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"1282","DOI":"10.1097\/PAS.0000000000001315","article-title":"Application of a 5 Marker Panel to the Routine Diagnosis of Peripheral T-Cell Lymphoma with T-Follicular Helper Phenotype","volume":"43","author":"Basha","year":"2019","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"678","DOI":"10.1002\/cam4.2742","article-title":"EBV status has prognostic implication among young patients with angioimmunoblastic T-cell lymphoma","volume":"9","author":"Eladl","year":"2020","journal-title":"Cancer Med."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"368","DOI":"10.1309\/6UTX-GVC0-12ND-JJEU","article-title":"Epstein-Barr virus-associated B-cell lymphoproliferative disorders in angloimmunoblastic T-cell lymphoma and peripheral T-cell lymphoma, unspecified","volume":"117","author":"Zettl","year":"2002","journal-title":"Am. J. Clin. Pathol."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"1789","DOI":"10.1182\/blood.V79.7.1789.1789","article-title":"Detection and localization of Epstein-Barr viral genomes in angioimmunoblastic lymphadenopathy and angioimmunoblastic lymphadenopathy-like lymphoma","volume":"79","author":"Weiss","year":"1992","journal-title":"Blood"},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"171","DOI":"10.1038\/ng.2872","article-title":"Somatic RHOA mutation in angioimmunoblastic T cell lymphoma","volume":"46","author":"Enami","year":"2014","journal-title":"Nat. Genet."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"166","DOI":"10.1038\/ng.2873","article-title":"Recurrent mutations in epigenetic regulators, RHOA and FYN kinase in peripheral T cell lymphomas","volume":"46","author":"Palomero","year":"2014","journal-title":"Nat. Genet."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"1466","DOI":"10.1182\/blood-2012-02-408542","article-title":"Recurrent TET2 mutations in peripheral T-cell lymphomas correlate with TFH-like features and adverse clinical parameters","volume":"120","author":"Lemonnier","year":"2012","journal-title":"Blood"},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"1901","DOI":"10.1182\/blood-2011-11-391748","article-title":"IDH2 mutations are frequent in angioimmunoblastic T-cell lymphoma","volume":"119","author":"Cairns","year":"2012","journal-title":"Blood"},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"15084","DOI":"10.1073\/pnas.1617929114","article-title":"The IDH2 R172K mutation associated with angioimmunoblastic T-cell lymphoma produces 2HG in T cells and impacts lymphoid development","volume":"113","author":"Lemonnier","year":"2016","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"1062","DOI":"10.1038\/leu.2015.357","article-title":"Recurrent activating mutations of CD28 in peripheral T-cell lymphomas","volume":"30","author":"Rohr","year":"2016","journal-title":"Leukemia"},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"1490","DOI":"10.1182\/blood-2016-02-698977","article-title":"Activating mutations in genes related to TCR signaling in angioimmunoblastic and other follicular helper T-cell-derived lymphomas","volume":"128","author":"Vallois","year":"2016","journal-title":"Blood"},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"694","DOI":"10.1038\/leu.2017.273","article-title":"Activation of RHOA-VAV1 signaling in angioimmunoblastic T-cell lymphoma","volume":"32","author":"Fujisawa","year":"2018","journal-title":"Leukemia"},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"2477","DOI":"10.1056\/NEJMoa1409405","article-title":"Clonal hematopoiesis and blood-cancer risk inferred from blood DNA sequence","volume":"371","author":"Genovese","year":"2014","journal-title":"N. Engl. J. Med."},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"2488","DOI":"10.1056\/NEJMoa1408617","article-title":"Age-related clonal hematopoiesis associated with adverse outcomes","volume":"371","author":"Jaiswal","year":"2014","journal-title":"N. Engl. J. Med."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"523","DOI":"10.1007\/s12185-015-1738-8","article-title":"Multistep tumorigenesis in peripheral T cell lymphoma","volume":"102","year":"2015","journal-title":"Int. J. Hematol."},{"key":"ref_54","first-page":"1123","article-title":"The pathological features of angioimmunoblastic T-cell lymphomas with IDH2(R172) mutations","volume":"32","author":"Steinhilber","year":"2019","journal-title":"Mod. Pathol. Off. J. USA Can. Acad. Pathol. Inc."},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"1041","DOI":"10.1097\/PAS.0000000000000651","article-title":"Clinicopathologic Analysis of Angioimmunoblastic T-cell Lymphoma with or without RHOA G17V Mutation Using Formalin-fixed Paraffin-embedded Sections","volume":"40","author":"Nagao","year":"2016","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"335","DOI":"10.1097\/PAS.0000000000000555","article-title":"Angioimmunoblastic T-cell Lymphomas with the RHOA p.Gly17Val Mutation Have Classic Clinical and Pathologic Features","volume":"40","author":"Ondrejka","year":"2016","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"682","DOI":"10.1097\/PAS.0b013e3181971591","article-title":"Peripheral T-cell lymphomas with a follicular growth pattern are derived from follicular helper T cells (TFH) and may show overlapping features with angioimmunoblastic T-cell lymphomas","volume":"33","author":"Huang","year":"2009","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"879","DOI":"10.1309\/AJCPBPNV86VZENGV","article-title":"Clinicopathologic analysis of peripheral T-cell lymphoma, follicular variant, and comparison with angioimmunoblastic T-cell lymphoma: Bcl-6 expression might affect progression between these disorders","volume":"137","author":"Miyoshi","year":"2012","journal-title":"Am. J. Clin. Pathol."},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"395","DOI":"10.1097\/00000478-200103000-00015","article-title":"Peripheral T-cell lymphoma with follicular involvement and a CD4+\/bcl-6+ phenotype","volume":"25","author":"Savilo","year":"2001","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"78","DOI":"10.1007\/s00428-005-0123-z","article-title":"Peripheral T-cell lymphoma with involvement of the expanded mantle zone","volume":"449","author":"Ikonomou","year":"2006","journal-title":"Virchows Arch. Int. J. Pathol."},{"key":"ref_61","first-page":"1173","article-title":"Peripheral T-cell lymphomas of follicular helper T-cell type frequently display an aberrant CD3(-\/dim)CD4(+) population by flow cytometry: An important clue to the diagnosis of a Hodgkin lymphoma mimic","volume":"29","author":"Alikhan","year":"2016","journal-title":"Mod. Pathol. Off. J. USA Can. Acad. Pathol. Inc."},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"e148","DOI":"10.3324\/haematol.2016.158428","article-title":"Integrative clinicopathological and molecular analyses of angioimmunoblastic T-cell lymphoma and other nodal lymphomas of follicular helper T-cell origin","volume":"102","author":"Dobay","year":"2017","journal-title":"Haematologica"},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"584","DOI":"10.1182\/blood.2019002401","article-title":"FER and FES tyrosine kinase fusions in follicular T-cell lymphoma","volume":"135","author":"Debackere","year":"2020","journal-title":"Blood"},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"653","DOI":"10.1111\/pin.12981","article-title":"RHOA mutation in follicular T-cell lymphoma: Clinicopathological analysis of 16 cases","volume":"70","author":"Miyoshi","year":"2020","journal-title":"Pathol. Int."},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"313","DOI":"10.1038\/sj.leu.2404045","article-title":"Novel t(5; 9)(q33; q22) fuses ITK to SYK in unspecified peripheral T-cell lymphoma","volume":"20","author":"Streubel","year":"2006","journal-title":"Leukemia"},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"506","DOI":"10.1097\/PAS.0000000000000813","article-title":"Clinicopathological Study of 30 Cases of Peripheral T-cell Lymphoma with Hodgkin and Reed-Sternberg-like B-cells from Japan","volume":"41","author":"Eladl","year":"2017","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"816","DOI":"10.1097\/PAS.0b013e3182785610","article-title":"Peripheral T-cell lymphomas of follicular T-helper cell derivation with Hodgkin\/Reed-Sternberg cells of B-cell lineage: Both EBV-positive and EBV-negative variants exist","volume":"37","author":"Nicolae","year":"2013","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"1233","DOI":"10.1097\/00000478-199910000-00008","article-title":"Peripheral T-cell lymphoma with Reed-Sternberg-like cells of B-cell phenotype and genotype associated with Epstein-Barr virus infection","volume":"23","author":"Fend","year":"1999","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"1156","DOI":"10.1111\/his.13475","article-title":"Immunohistochemical assessment of the diagnostic utility of PD-L1: A preliminary analysis of anti-PD-L1 antibody (SP142) for lymphoproliferative diseases with tumour and non-malignant Hodgkin-Reed-Sternberg (HRS)-like cells","volume":"72","author":"Sakakibara","year":"2018","journal-title":"Histopathology"},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"319","DOI":"10.1002\/(SICI)1097-0215(20000201)85:3<319::AID-IJC4>3.0.CO;2-B","article-title":"Evaluation of adult T-cell leukemia\/lymphoma incidence and its impact on non-Hodgkin lymphoma incidence in southwestern Japan","volume":"85","author":"Arisawa","year":"2000","journal-title":"Int. J. Cancer"},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"237","DOI":"10.1002\/ijc.2910450206","article-title":"The 4th nation-wide study of adult T-cell leukemia\/lymphoma (ATL) in Japan: Estimates of risk of ATL and its geographical and clinical features. The T- and B-cell Malignancy Study Group","volume":"45","author":"Tajima","year":"1990","journal-title":"Int. J. Cancer"},{"key":"ref_72","first-page":"226","article-title":"Incidence of adult T-cell leukemia\/lymphoma among human T-lymphotropic virus type I carriers in Saga, Japan","volume":"49","author":"Tokudome","year":"1989","journal-title":"Cancer Res."},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"270","DOI":"10.1038\/nrc2111","article-title":"Human T-cell leukaemia virus type 1 (HTLV-1) infectivity and cellular transformation","volume":"7","author":"Matsuoka","year":"2007","journal-title":"Nat. Rev. Cancer"},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"428","DOI":"10.1111\/j.1365-2141.1991.tb08051.x","article-title":"Diagnostic criteria and classification of clinical subtypes of adult T-cell leukaemia-lymphoma. A report from the Lymphoma Study Group (1984-87)","volume":"79","author":"Shimoyama","year":"1991","journal-title":"Br. J. Haematol."},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"4346","DOI":"10.1111\/cas.15097","article-title":"Epidemiology of adult T-cell leukemia-lymphoma in Japan: An updated analysis, 2012\u20132013","volume":"112","author":"Ito","year":"2021","journal-title":"Cancer Sci."},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"2570","DOI":"10.1182\/blood-2015-03-632489","article-title":"Treatment and survival among 1594 patients with ATL","volume":"126","author":"Katsuya","year":"2015","journal-title":"Blood"},{"key":"ref_77","doi-asserted-by":"crossref","first-page":"453","DOI":"10.1200\/JCO.2008.18.2428","article-title":"Definition, prognostic factors, treatment, and response criteria of adult T-cell leukemia-lymphoma: A proposal from an international consensus meeting","volume":"27","author":"Tsukasaki","year":"2009","journal-title":"J. Clin. Oncol. Off. J. Am. Soc. Clin. Oncol."},{"key":"ref_78","doi-asserted-by":"crossref","first-page":"216","DOI":"10.1097\/01.pas.0000213325.79368.2c","article-title":"Adult T-cell lymphoma\/leukemia with angioimmunoblastic T-cell lymphomalike features: Report of 11 cases","volume":"31","author":"Karube","year":"2007","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_79","first-page":"3625","article-title":"Clinical significance of CCR4 expression in adult T-cell leukemia\/lymphoma: Its close association with skin involvement and unfavorable outcome","volume":"9","author":"Ishida","year":"2003","journal-title":"Clin. Cancer Res. Off. J. Am. Assoc. Cancer Res."},{"key":"ref_80","doi-asserted-by":"crossref","first-page":"81","DOI":"10.1111\/j.1365-2141.2004.04999.x","article-title":"Expression of FoxP3, a key molecule in CD4CD25 regulatory T cells, in adult T-cell leukaemia\/lymphoma cells","volume":"126","author":"Karube","year":"2004","journal-title":"Br. J. Haematol."},{"key":"ref_81","first-page":"617","article-title":"The relationship of FOXP3 expression and clinicopathological characteristics in adult T-cell leukemia\/lymphoma","volume":"21","author":"Karube","year":"2008","journal-title":"Mod. Pathol. Off. J. USA Can. Acad. Pathol. Inc."},{"key":"ref_82","doi-asserted-by":"crossref","first-page":"592","DOI":"10.1002\/(SICI)1097-0215(19970807)72:4<592::AID-IJC7>3.0.CO;2-L","article-title":"Clonal HTLV-I-infected CD4+ T-lymphocytes and non-clonal non-HTLV-I-infected giant cells in incipient ATLL with Hodgkin-like histologic features","volume":"72","author":"Ohshima","year":"1997","journal-title":"Int. J. Cancer"},{"key":"ref_83","doi-asserted-by":"crossref","first-page":"1042","DOI":"10.1016\/j.humpath.2010.10.014","article-title":"Adult T-cell leukemia\/lymphoma with Epstein-Barr virus-positive Hodgkin-like cells","volume":"42","author":"Venkataraman","year":"2011","journal-title":"Hum. Pathol."},{"key":"ref_84","first-page":"198","article-title":"Clinicopathological features of adult T-cell leukemia\/lymphoma with HTLV-1-infected Hodgkin and Reed-Sternberg-like cells","volume":"5","author":"Karube","year":"2021","journal-title":"Blood Adv."},{"key":"ref_85","doi-asserted-by":"crossref","first-page":"1304","DOI":"10.1038\/ng.3415","article-title":"Integrated molecular analysis of adult T cell leukemia\/lymphoma","volume":"47","author":"Kataoka","year":"2015","journal-title":"Nat. Genet."},{"key":"ref_86","doi-asserted-by":"crossref","first-page":"1507","DOI":"10.1182\/blood-2018-01-824607","article-title":"North American ATLL has a distinct mutational and transcriptional profile and responds to epigenetic therapies","volume":"132","author":"Shah","year":"2018","journal-title":"Blood"},{"key":"ref_87","doi-asserted-by":"crossref","first-page":"1719","DOI":"10.1111\/cas.13303","article-title":"Genetic alterations in adult T-cell leukemia\/lymphoma","volume":"108","author":"Kogure","year":"2017","journal-title":"Cancer Sci."},{"key":"ref_88","doi-asserted-by":"crossref","unstructured":"Yoshida, N., Miyoshi, H., and Ohshima, K. (2021). Clinical Applications of Genomic Alterations in ATLL: Predictive Markers and Therapeutic Targets. Cancers, 13.","DOI":"10.3390\/cancers13081801"},{"key":"ref_89","doi-asserted-by":"crossref","first-page":"1467","DOI":"10.1182\/blood.2019001815","article-title":"Genomic landscape of young ATLL patients identifies frequent targetable CD28 fusions","volume":"135","author":"Yoshida","year":"2020","journal-title":"Blood"},{"key":"ref_90","doi-asserted-by":"crossref","first-page":"402","DOI":"10.1038\/nature18294","article-title":"Aberrant PD-L1 expression through 3\u2019-UTR disruption in multiple cancers","volume":"534","author":"Kataoka","year":"2016","journal-title":"Nature"},{"key":"ref_91","doi-asserted-by":"crossref","first-page":"6129","DOI":"10.1158\/0008-5472.CAN-14-0643","article-title":"Molecular characterization of chronic-type adult T-cell leukemia\/lymphoma","volume":"74","author":"Yoshida","year":"2014","journal-title":"Cancer Res."},{"key":"ref_92","doi-asserted-by":"crossref","first-page":"1374","DOI":"10.1182\/blood-2016-02-698936","article-title":"PD-L1 expression on neoplastic or stromal cells is respectively a poor or good prognostic factor for adult T-cell leukemia\/lymphoma","volume":"128","author":"Miyoshi","year":"2016","journal-title":"Blood"},{"key":"ref_93","doi-asserted-by":"crossref","first-page":"1626","DOI":"10.3324\/haematol.2018.205567","article-title":"Human leukocyte antigen class II expression is a good prognostic factor in adult T-cell leukemia\/lymphoma","volume":"104","author":"Takeuchi","year":"2019","journal-title":"Haematologica"},{"key":"ref_94","doi-asserted-by":"crossref","first-page":"621","DOI":"10.1002\/path.4699","article-title":"CCR4 frameshift mutation identifies a distinct group of adult T cell leukaemia\/lymphoma with poor prognosis","volume":"238","author":"Yoshida","year":"2016","journal-title":"J. Pathol."},{"key":"ref_95","doi-asserted-by":"crossref","first-page":"2497","DOI":"10.1084\/jem.20140987","article-title":"Gain-of-function CCR4 mutations in adult T cell leukemia\/lymphoma","volume":"211","author":"Nakagawa","year":"2014","journal-title":"J. Exp. Med."},{"key":"ref_96","doi-asserted-by":"crossref","first-page":"758","DOI":"10.1182\/blood-2018-02-835991","article-title":"CCR4 mutations associated with superior outcome of adult T-cell leukemia\/lymphoma under mogamulizumab treatment","volume":"132","author":"Sakamoto","year":"2018","journal-title":"Blood"},{"key":"ref_97","doi-asserted-by":"crossref","first-page":"215","DOI":"10.1182\/blood-2017-01-761874","article-title":"Prognostic relevance of integrated genetic profiling in adult T-cell leukemia\/lymphoma","volume":"131","author":"Kataoka","year":"2018","journal-title":"Blood"},{"key":"ref_98","doi-asserted-by":"crossref","first-page":"159","DOI":"10.1182\/blood-2017-11-815472","article-title":"Clinicogenetic risk modeling in ATL","volume":"131","author":"Yoshida","year":"2018","journal-title":"Blood"},{"key":"ref_99","doi-asserted-by":"crossref","first-page":"3402","DOI":"10.1182\/blood-2010-09-310342","article-title":"Peripheral T-cell lymphoma, not otherwise specified: A report of 340 cases from the International Peripheral T-cell Lymphoma Project","volume":"117","author":"Weisenburger","year":"2011","journal-title":"Blood"},{"key":"ref_100","doi-asserted-by":"crossref","first-page":"334","DOI":"10.1007\/s00428-004-1077-2","article-title":"Nodal peripheral T-cell lymphomas and, in particular, their lymphoepithelioid (Lennert\u2019s) variant are often derived from CD8(+) cytotoxic T-cells","volume":"445","author":"Geissinger","year":"2004","journal-title":"Virchows Arch. Int. J. Pathol."},{"key":"ref_101","doi-asserted-by":"crossref","first-page":"1627","DOI":"10.1097\/00000478-200012000-00006","article-title":"Lennert\u2019s lymphoma: A variant of cytotoxic T-cell lymphoma?","volume":"24","author":"Yamashita","year":"2000","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_102","doi-asserted-by":"crossref","first-page":"517","DOI":"10.1002\/1097-0142(19880201)61:3<517::AID-CNCR2820610317>3.0.CO;2-1","article-title":"Lennert\u2019s lymphoma. A clinicopathologic study with emphasis on phenotype and its relationship to survival","volume":"61","author":"Spier","year":"1988","journal-title":"Cancer"},{"key":"ref_103","doi-asserted-by":"crossref","first-page":"2472","DOI":"10.1200\/JCO.2005.03.6327","article-title":"Marker expression in peripheral T-cell lymphoma: A proposed clinical-pathologic prognostic score","volume":"24","author":"Went","year":"2006","journal-title":"J. Clin. Oncol. Off. J. Am. Soc. Clin. Oncol."},{"key":"ref_104","doi-asserted-by":"crossref","first-page":"1284","DOI":"10.1097\/01.pas.0000173238.17331.6b","article-title":"Clinicopathologic and prognostic significance of cytotoxic molecule expression in nodal peripheral T-cell lymphoma, unspecified","volume":"29","author":"Asano","year":"2005","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_105","doi-asserted-by":"crossref","first-page":"2599","DOI":"10.1111\/cas.13652","article-title":"Reappraisal of nodal Epstein-Barr Virus-negative cytotoxic T-cell lymphoma: Identification of indolent CD5(+) diseases","volume":"109","author":"Yamashita","year":"2018","journal-title":"Cancer Sci."},{"key":"ref_106","doi-asserted-by":"crossref","first-page":"186","DOI":"10.1111\/j.1365-2559.2012.04199.x","article-title":"Nodal cytotoxic molecule (CM)-positive Epstein-Barr virus (EBV)-associated peripheral T cell lymphoma (PTCL): A clinicopathological study of 26 cases","volume":"61","author":"Kato","year":"2012","journal-title":"Histopathology"},{"key":"ref_107","doi-asserted-by":"crossref","first-page":"462","DOI":"10.1097\/PAS.0000000000000323","article-title":"T-cell receptor (TCR) phenotype of nodal Epstein-Barr virus (EBV)-positive cytotoxic T-cell lymphoma (CTL): A clinicopathologic study of 39 cases","volume":"39","author":"Kato","year":"2015","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_108","doi-asserted-by":"crossref","first-page":"30","DOI":"10.3960\/jslrt.20001","article-title":"Nodal EBV+ cytotoxic T-cell lymphoma: A literature review based on the 2017 WHO classification","volume":"60","author":"Kato","year":"2020","journal-title":"J. Clin. Exp. Hematop."},{"key":"ref_109","doi-asserted-by":"crossref","first-page":"2915","DOI":"10.1182\/blood-2013-11-536359","article-title":"Gene expression signatures delineate biological and prognostic subgroups in peripheral T-cell lymphoma","volume":"123","author":"Iqbal","year":"2014","journal-title":"Blood"},{"key":"ref_110","doi-asserted-by":"crossref","first-page":"1664","DOI":"10.1182\/blood-2018-09-872549","article-title":"Genetic drivers of oncogenic pathways in molecular subgroups of peripheral T-cell lymphoma","volume":"133","author":"Heavican","year":"2019","journal-title":"Blood"},{"key":"ref_111","doi-asserted-by":"crossref","first-page":"2159","DOI":"10.1182\/blood.2019000779","article-title":"Reproducing the molecular subclassification of peripheral T-cell lymphoma-NOS by immunohistochemistry","volume":"134","author":"Amador","year":"2019","journal-title":"Blood"},{"key":"ref_112","doi-asserted-by":"crossref","first-page":"2867","DOI":"10.1038\/s41375-019-0473-1","article-title":"Molecular heterogeneity in peripheral T-cell lymphoma, not otherwise specified revealed by comprehensive genetic profiling","volume":"33","author":"Watatani","year":"2019","journal-title":"Leukemia"},{"key":"ref_113","doi-asserted-by":"crossref","first-page":"2918","DOI":"10.3324\/haematol.2020.262659","article-title":"CDKN2A deletion is a frequent event associated with poor outcome in patients with peripheral T-cell lymphoma not otherwise specified (PTCL-NOS)","volume":"106","author":"Maura","year":"2021","journal-title":"Haematologica"},{"key":"ref_114","first-page":"179","article-title":"Whole exome sequencing reveals mutations in FAT1 tumor suppressor gene clinically impacting on peripheral T-cell lymphoma not otherwise specified","volume":"33","author":"Laginestra","year":"2020","journal-title":"Mod. Pathol. Off. J. USA Can. Acad. Pathol. Inc."},{"key":"ref_115","doi-asserted-by":"crossref","first-page":"2242","DOI":"10.1182\/bloodadvances.2018018754","article-title":"Microenvironmental immune cell signatures dictate clinical outcomes for PTCL-NOS","volume":"2","author":"Sugio","year":"2018","journal-title":"Blood Adv."}],"container-title":["Diagnostics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2075-4418\/12\/8\/2001\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T00:11:46Z","timestamp":1760141506000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2075-4418\/12\/8\/2001"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,8,18]]},"references-count":115,"journal-issue":{"issue":"8","published-online":{"date-parts":[[2022,8]]}},"alternative-id":["diagnostics12082001"],"URL":"https:\/\/doi.org\/10.3390\/diagnostics12082001","relation":{},"ISSN":["2075-4418"],"issn-type":[{"value":"2075-4418","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,8,18]]}}}