{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,28]],"date-time":"2026-01-28T12:19:33Z","timestamp":1769602773109,"version":"3.49.0"},"reference-count":75,"publisher":"BMJ","issue":"12","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["J Clin Pathol"],"accepted":{"date-parts":[[2015,11,5]]},"published-print":{"date-parts":[[2015,12]]},"abstract":"<jats:p>Primary cutaneous T-cell lymphomas (CTCLs) represent a number of extranodal lymphomas arising from a malignant population of lymphocytes in the skin, with the most common type being mycosis fungoides (MF) representing half of all primary CTCLs. Despite advances in immunohistochemistry and molecular methodology, significant diagnostic challenges remain due to phenotypic overlap of primary CTCLs with several inflammatory dermatoses, secondary lymphomas, among other conditions. Clinical features such as presentation and morphology, staging, histology, immunophenotype and molecular features must be considered in detail before a diagnosis is made in order to minimise false-positive, false-negative and indeterminate diagnoses. Herein, we review primary CTCLs, including epidemiological data, a brief summary of clinical presentations, immunophenotype, molecular signatures and differential diagnoses.<\/jats:p>","DOI":"10.1136\/jclinpath-2015-203133","type":"journal-article","created":{"date-parts":[[2015,11,24]],"date-time":"2015-11-24T00:52:40Z","timestamp":1448326360000},"page":"1003-1010","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":24,"title":["Primary cutaneous T-cell lymphomas: a review"],"prefix":"10.1136","volume":"68","author":[{"given":"Konstantinos G","family":"Sidiropoulos","sequence":"first","affiliation":[{"name":"Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M Estela","family":"Martinez-Escala","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Northwestern University, Feinberg School of Medicine, Chicago, Illinois, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Oriol","family":"Yelamos","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Northwestern University, Feinberg School of Medicine, Chicago, Illinois, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Joan","family":"Guitart","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Northwestern University, Feinberg School of Medicine, Chicago, Illinois, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael","family":"Sidiropoulos","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Canada"},{"name":"Department of Pathology, Lakeridge Health, Oshawa, Ontario, Canada"},{"name":"Department of Pathology and Laboratory Medicine, University Health Network, Toronto, Ontario, Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2015,11,23]]},"reference":[{"key":"2025112110201367000_68.12.1003.1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2004-09-3502"},{"key":"2025112110201367000_68.12.1003.2","doi-asserted-by":"crossref","unstructured":"Cerroni L . Skin lymphoma the illustrated guide. Chichester, West Sussex, UK; Hoboken, NJ: Wiley-Blackwell, 2009:x, 276 p.","DOI":"10.1002\/9781444316742"},{"key":"2025112110201367000_68.12.1003.3","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2013.06.012"},{"key":"2025112110201367000_68.12.1003.4","unstructured":"Swerdlow SH , International Agency for Research on Cancer., and World Health Organization. WHO classification of tumours of haematopoietic and lymphoid tissues. 4th edn. World Health Organization classification of tumours. Lyon, France: International Agency for Research on Cancer, 2008: 439p."},{"key":"2025112110201367000_68.12.1003.5","unstructured":"Hall JC , Hall BJ . Cutaneous lymphoma: diagnosis and treatment. Shelton, Conn.: People's Medical Pub. House-USA, 2012:xv, 248 p."},{"key":"2025112110201367000_68.12.1003.6","doi-asserted-by":"publisher","DOI":"10.1586\/17474086.2014.942279"},{"key":"2025112110201367000_68.12.1003.7","doi-asserted-by":"publisher","DOI":"10.1001\/archdermatol.2008.567"},{"key":"2025112110201367000_68.12.1003.8","doi-asserted-by":"publisher","DOI":"10.1001\/archderm.143.7.921"},{"key":"2025112110201367000_68.12.1003.9","doi-asserted-by":"publisher","DOI":"10.5858\/arpa.2014-0298-CC"},{"key":"2025112110201367000_68.12.1003.10","doi-asserted-by":"publisher","DOI":"10.1111\/j.1468-3083.2004.00937.x"},{"key":"2025112110201367000_68.12.1003.11","doi-asserted-by":"publisher","DOI":"10.1034\/j.1600-0560.2001.028004174.x"},{"key":"2025112110201367000_68.12.1003.12","doi-asserted-by":"publisher","DOI":"10.1097\/00000478-199512000-00009"},{"key":"2025112110201367000_68.12.1003.13","doi-asserted-by":"publisher","DOI":"10.1097\/01.pas.0000153121.57515.c6"},{"key":"2025112110201367000_68.12.1003.14","doi-asserted-by":"crossref","first-page":"276","DOI":"10.1097\/DAD.0b013e3181c89e8c","article-title":"Histopathological changes seen in mycosis fungoides patients after phototherapy","volume":"32","author":"Apa","year":"2010","journal-title":"Am J Dermatopathol"},{"key":"2025112110201367000_68.12.1003.15","doi-asserted-by":"publisher","DOI":"10.1309\/U1Y6-J4AG-5M4M-7AYV"},{"key":"2025112110201367000_68.12.1003.16","doi-asserted-by":"publisher","DOI":"10.1097\/01.MP.0000084112.81779.BB"},{"key":"2025112110201367000_68.12.1003.17","first-page":"62","article-title":"Immunohistological markers for the diagnosis of cutaneous lymphomas","volume":"8","author":"Ralfkiaer","year":"1991","journal-title":"Semin Diagn Pathol"},{"key":"2025112110201367000_68.12.1003.18","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2133.2005.06649.x"},{"key":"2025112110201367000_68.12.1003.19","doi-asserted-by":"publisher","DOI":"10.1002\/1097-0142(19911001)68:7<1572::AID-CNCR2820680719>3.0.CO;2-1"},{"key":"2025112110201367000_68.12.1003.20","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2133.2002.04966.x"},{"key":"2025112110201367000_68.12.1003.21","doi-asserted-by":"crossref","first-page":"3371","DOI":"10.1182\/blood.V89.9.3371","article-title":"Recurring structural chromosome abnormalities in peripheral blood lymphocytes of patients with mycosis fungoides\/Sezary syndrome","volume":"89","author":"Thangavelu","year":"1997","journal-title":"Blood"},{"key":"2025112110201367000_68.12.1003.22","doi-asserted-by":"crossref","first-page":"2937","DOI":"10.1182\/blood.V95.9.2937.009k15_2937_2942","article-title":"Loss of heterozygosity on 10q and microsatellite instability in advanced stages of primary cutaneous T-cell lymphoma and possible association with homozygous deletion of PTEN","volume":"95","author":"Scarisbrick","year":"2000","journal-title":"Blood"},{"key":"2025112110201367000_68.12.1003.23","doi-asserted-by":"publisher","DOI":"10.1016\/j.humpath.2006.09.012"},{"key":"2025112110201367000_68.12.1003.24","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2002-11-3574"},{"key":"2025112110201367000_68.12.1003.25","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":"2025112110201367000_68.12.1003.26","doi-asserted-by":"publisher","DOI":"10.1001\/archdermatol.2009.74"},{"key":"2025112110201367000_68.12.1003.27","doi-asserted-by":"crossref","first-page":"1343","DOI":"10.1200\/JCO.1995.13.6.1343","article-title":"CD30\/Ki-1-positive lymphoproliferative disorders of the skin--clinicopathologic correlation and statistical analysis of 86 cases: a multicentric study from the European Organization for Research and Treatment of Cancer Cutaneous Lymphoma Project Group","volume":"13","author":"Paulli","year":"1995","journal-title":"J Clin Oncol"},{"key":"2025112110201367000_68.12.1003.28","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2133.2003.05248.x"},{"key":"2025112110201367000_68.12.1003.29","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2559.2000.00799.x"},{"key":"2025112110201367000_68.12.1003.30","doi-asserted-by":"publisher","DOI":"10.1111\/1523-1747.ep12337633"},{"key":"2025112110201367000_68.12.1003.31","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2559.1993.tb00470.x"},{"key":"2025112110201367000_68.12.1003.32","doi-asserted-by":"crossref","first-page":"3437","DOI":"10.1182\/blood.V87.8.3437.bloodjournal8783437","article-title":"The t(2;5) chromosomal translocation is not a common feature of primary cutaneous CD30+ lymphoproliferative disorders: comparison with anaplastic large-cell lymphoma of nodal origin","volume":"87","author":"Decoteau","year":"1996","journal-title":"Blood"},{"key":"2025112110201367000_68.12.1003.33","doi-asserted-by":"publisher","DOI":"10.1136\/jcp.53.6.445"},{"key":"2025112110201367000_68.12.1003.34","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2004-03-1037"},{"key":"2025112110201367000_68.12.1003.35","doi-asserted-by":"publisher","DOI":"10.1038\/sj.leu.2401878"},{"key":"2025112110201367000_68.12.1003.36","unstructured":"Swerdlow SH , Organizaci\u00f3n Mundial de la Salud., and Agencia Internacional de Investigacion sobre el C\u00e1ncer. WHO classification of tumours of hematopoietic and lymphoid tissues. 4th edn. World Health Organization classification of tumours, Lyon: International Agency for Research on Cancer, 2008:439p."},{"key":"2025112110201367000_68.12.1003.37","doi-asserted-by":"publisher","DOI":"10.1053\/j.semdp.2009.09.001"},{"key":"2025112110201367000_68.12.1003.38","doi-asserted-by":"publisher","DOI":"10.1016\/S0190-9622(03)02484-8"},{"key":"2025112110201367000_68.12.1003.39","first-page":"392","article-title":"Essential thrombocythemia with ringed sideroblasts: a heterogeneous spectrum of diseases, but not a distinct entity","volume":"87","author":"Schmitt-Graeff","year":"2002","journal-title":"Haematologica"},{"key":"2025112110201367000_68.12.1003.40","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2133.2001.04025.x"},{"issue":"1","key":"2025112110201367000_68.12.1003.41","doi-asserted-by":"crossref","first-page":"10","DOI":"10.1111\/j.0303-6987.2006.00507.x","article-title":"Pathobiology of CD30+ cutaneous T-cell lymphomas","volume":"33","author":"Kadin","year":"2006","journal-title":"J Cutan Pathol"},{"key":"2025112110201367000_68.12.1003.42","doi-asserted-by":"publisher","DOI":"10.1111\/bjd.13364"},{"key":"2025112110201367000_68.12.1003.43","doi-asserted-by":"publisher","DOI":"10.1097\/DAD.0b013e31825ba953"},{"key":"2025112110201367000_68.12.1003.44","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0b013e3182648596"},{"key":"2025112110201367000_68.12.1003.45","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2007-04-087288"},{"key":"2025112110201367000_68.12.1003.46","doi-asserted-by":"publisher","DOI":"10.1111\/j.0303-6987.2006.00493.x"},{"key":"2025112110201367000_68.12.1003.47","doi-asserted-by":"publisher","DOI":"10.1016\/S0046-8177(98)90122-8"},{"key":"2025112110201367000_68.12.1003.48","doi-asserted-by":"publisher","DOI":"10.1097\/00000478-199807000-00010"},{"key":"2025112110201367000_68.12.1003.49","first-page":"1723","article-title":"Nonhepatosplenic gammadelta T-cell lymphoma: a subset of cytotoxic lymphomas with mucosal or skin localization","volume":"91","author":"Arnulf","year":"1998","journal-title":"Blood"},{"key":"2025112110201367000_68.12.1003.50","doi-asserted-by":"publisher","DOI":"10.1001\/archderm.136.8.1024"},{"key":"2025112110201367000_68.12.1003.51","doi-asserted-by":"publisher","DOI":"10.1016\/0190-9622(90)70074-R"},{"key":"2025112110201367000_68.12.1003.52","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9440(10)65144-9"},{"key":"2025112110201367000_68.12.1003.53","doi-asserted-by":"publisher","DOI":"10.1002\/cncr.11107"},{"key":"2025112110201367000_68.12.1003.54","doi-asserted-by":"publisher","DOI":"10.1159\/000087025"},{"key":"2025112110201367000_68.12.1003.55","doi-asserted-by":"publisher","DOI":"10.1097\/00000372-199506000-00012"},{"key":"2025112110201367000_68.12.1003.56","doi-asserted-by":"publisher","DOI":"10.1097\/01.pas.0000126719.71954.4f"},{"key":"2025112110201367000_68.12.1003.57","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2002-07-1960"},{"key":"2025112110201367000_68.12.1003.58","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2002-05-1597"},{"key":"2025112110201367000_68.12.1003.59","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.cb.11.110195.001515"},{"key":"2025112110201367000_68.12.1003.60","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2559.2000.00893.x"},{"key":"2025112110201367000_68.12.1003.61","doi-asserted-by":"publisher","DOI":"10.1016\/S0037-1963(03)00132-X"},{"key":"2025112110201367000_68.12.1003.62","doi-asserted-by":"publisher","DOI":"10.1111\/1523-1747.ep12470953"},{"key":"2025112110201367000_68.12.1003.63","doi-asserted-by":"publisher","DOI":"10.1097\/00000478-200202000-00009"},{"key":"2025112110201367000_68.12.1003.64","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2133.1996.d01-1106.x"},{"key":"2025112110201367000_68.12.1003.65","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-0625.1992.tb00069.x"},{"key":"2025112110201367000_68.12.1003.66","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0b013e31826a5038"},{"key":"2025112110201367000_68.12.1003.67","doi-asserted-by":"publisher","DOI":"10.1016\/S1525-1578(10)60610-1"},{"key":"2025112110201367000_68.12.1003.68","doi-asserted-by":"publisher","DOI":"10.3109\/10428199509064919"},{"key":"2025112110201367000_68.12.1003.69","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2133.1992.tb15116.x"},{"key":"2025112110201367000_68.12.1003.70","doi-asserted-by":"publisher","DOI":"10.1001\/archderm.1995.01690210039007"},{"key":"2025112110201367000_68.12.1003.71","doi-asserted-by":"publisher","DOI":"10.1067\/mjd.2002.119663"},{"key":"2025112110201367000_68.12.1003.72","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.1998.00977.x"},{"key":"2025112110201367000_68.12.1003.73","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2008.16.1307"},{"key":"2025112110201367000_68.12.1003.74","doi-asserted-by":"publisher","DOI":"10.1002\/path.1711720110"},{"key":"2025112110201367000_68.12.1003.75","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0b013e318068b527"}],"container-title":["Journal of Clinical Pathology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/jclinpath-2015-203133","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,11,21]],"date-time":"2025-11-21T18:20:27Z","timestamp":1763749227000},"score":1,"resource":{"primary":{"URL":"https:\/\/jcp.bmj.com\/lookup\/doi\/10.1136\/jclinpath-2015-203133"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2015,11,23]]},"references-count":75,"journal-issue":{"issue":"12","published-online":{"date-parts":[[2015,11,23]]},"published-print":{"date-parts":[[2015,12]]}},"alternative-id":["10.1136\/jclinpath-2015-203133"],"URL":"https:\/\/doi.org\/10.1136\/jclinpath-2015-203133","relation":{},"ISSN":["0021-9746","1472-4146"],"issn-type":[{"value":"0021-9746","type":"print"},{"value":"1472-4146","type":"electronic"}],"subject":[],"published":{"date-parts":[[2015,11,23]]}}}