{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,4]],"date-time":"2026-10-04T01:55:13Z","timestamp":1791078913964,"version":"4.1.0"},"reference-count":9,"publisher":"Wiley","license":[{"start":{"date-parts":[[2021,6,1]],"date-time":"2021-06-01T00:00:00Z","timestamp":1622505600000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Case Reports in Hematology"],"published-print":{"date-parts":[[2021,6,1]]},"abstract":"<jats:p>S\u00e9zary syndrome is a rare leukemic type of cutaneous T-cell lymphoma characterized by the presence of neoplastic T cells with cerebriform nuclei (S\u00e9zary cells) in the skin, lymph nodes, and peripheral blood. Typical S\u00e9zary cells have a CD3+CD4+CD8\u2013 phenotype; however, in cases of the aberrant loss of antigens on S\u00e9zary cells, especially the loss of critically important T-cell antigens such as CD4, there is a possibility of misdiagnosing the disease or underestimating the tumor burden of the disease. Here, we report a rare case of S\u00e9zary syndrome with CD4\/CD8 double-negative S\u00e9zary cells in the peripheral blood. Most of the S\u00e9zary cells in the peripheral blood had lost CD4 expression, and we diagnosed the disease and evaluated the tumor burden by multicolor flow cytometry. Intriguingly, the S\u00e9zary cells showed a typical CD4+CD8\u2013CD7\u2013 phenotype in the skin even though the cells in the peripheral blood lacked CD4. The patient responded well to treatment with bexarotene and narrow-band ultraviolet B therapy. Analysis by multicolor flow cytometry is essential to diagnose this rare type of S\u00e9zary syndrome and evaluate the tumor burden.<\/jats:p>","DOI":"10.1155\/2021\/5527725","type":"journal-article","created":{"date-parts":[[2021,6,2]],"date-time":"2021-06-02T23:23:08Z","timestamp":1622676188000},"page":"1-5","source":"Crossref","is-referenced-by-count":1,"title":["S\u00e9zary Syndrome with CD4\/CD8 Double-Negative Neoplastic T Cells in Peripheral Blood"],"prefix":"10.1155","volume":"2021","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0564-2227","authenticated-orcid":true,"given":"Yuki","family":"Kageyama","sequence":"first","affiliation":[{"name":"Department of Hematology, Yokkaichi Municipal Hospital, 2-2-37 Shibata, Yokkaichi 510-8567, Japan"},{"name":"Department of Hematology and Oncology, Mie University Graduate School of Medicine, 2-174 Edobashi, Tsu 514-8507, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kenshiro","family":"Tsuda","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Yokkaichi Municipal Hospital, 2-2-37 Shibata, Yokkaichi 510-8567, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuma","family":"Nato","sequence":"additional","affiliation":[{"name":"Department of Hematology, Yokkaichi Municipal Hospital, 2-2-37 Shibata, Yokkaichi 510-8567, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6139-4256","authenticated-orcid":true,"given":"Keiki","family":"Nagaharu","sequence":"additional","affiliation":[{"name":"Department of Hematology and Oncology, Mie University Graduate School of Medicine, 2-174 Edobashi, Tsu 514-8507, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kazutaka","family":"Suzuki","sequence":"additional","affiliation":[{"name":"Department of Hematology, Yokkaichi Municipal Hospital, 2-2-37 Shibata, Yokkaichi 510-8567, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Akihiko","family":"Sawaki","sequence":"additional","affiliation":[{"name":"Department of Hematology, Yokkaichi Municipal Hospital, 2-2-37 Shibata, Yokkaichi 510-8567, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hiroyuki","family":"Miyashita","sequence":"additional","affiliation":[{"name":"Department of Hematology, Yokkaichi Municipal Hospital, 2-2-37 Shibata, Yokkaichi 510-8567, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","reference":[{"key":"1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2018-11-881268"},{"key":"2","doi-asserted-by":"publisher","DOI":"10.1309\/ajcp1na3ychcdeig"},{"key":"3","article-title":"Flow cytometric evaluation of peripheral blood for suspected S\u00e9zary syndrome or mycosis fungoides: International guidelines for assay characteristics","author":"P. Horna","year":"2020","journal-title":"Cytometry Part B: Clinical Cytometry"},{"key":"4","doi-asserted-by":"publisher","DOI":"10.1309\/ajcp09otjoyavzzk"},{"key":"5","doi-asserted-by":"publisher","DOI":"10.1038\/leu.2012.246"},{"key":"6","doi-asserted-by":"publisher","DOI":"10.1200\/jco.2010.32.0630"},{"key":"7","doi-asserted-by":"publisher","DOI":"10.1016\/j.jid.2016.01.038"},{"key":"8","doi-asserted-by":"crossref","first-page":"3766","DOI":"10.1200\/JCO.2015.61.7142","article-title":"Cutaneous lymphoma international consortium study of outcome in advanced stages of mycosis fungoides and S\u00e9zary syndrome: effect of specific prognostic markers on survival and development of a prognostic model","volume":"33","author":"J. J. Scarisbrick","year":"2015","journal-title":"Journal of Clinical Oncology"},{"key":"9","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2006.01.020"}],"container-title":["Case Reports in Hematology"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/downloads.hindawi.com\/journals\/crihem\/2021\/5527725.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/downloads.hindawi.com\/journals\/crihem\/2021\/5527725.xml","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/downloads.hindawi.com\/journals\/crihem\/2021\/5527725.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,6,2]],"date-time":"2021-06-02T23:23:12Z","timestamp":1622676192000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.hindawi.com\/journals\/crihem\/2021\/5527725\/"}},"subtitle":[],"editor":[{"given":"Kostas","family":"Konstantopoulos","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"editor"}]}],"short-title":[],"issued":{"date-parts":[[2021,6,1]]},"references-count":9,"alternative-id":["5527725","5527725"],"URL":"https:\/\/doi.org\/10.1155\/2021\/5527725","relation":{},"ISSN":["2090-6579","2090-6560"],"issn-type":[{"value":"2090-6579","type":"electronic"},{"value":"2090-6560","type":"print"}],"subject":[],"published":{"date-parts":[[2021,6,1]]}}}