{"id":2353,"date":"2015-01-15T12:20:58","date_gmt":"2015-01-15T12:20:58","guid":{"rendered":"https:\/\/medicalcriteria.com\/web\/sez\/"},"modified":"2026-04-06T19:34:10","modified_gmt":"2026-04-06T19:34:10","slug":"sez","status":"publish","type":"post","link":"https:\/\/medicalcriteria.com\/web\/sez\/","title":{"rendered":"Diagnostic Criteria in S\u00e9zary&#8217;s Syndrome (SS)"},"content":{"rendered":"<div class=\"99c380e4b4a7b96c35d7ddf7dcb434e8\" data-index=\"1\" style=\"float: none; margin:0px 0 0px 0; text-align:center;\">\n<script async src=\"https:\/\/pagead2.googlesyndication.com\/pagead\/js\/adsbygoogle.js\"><\/script>\r\n<!-- MC 2019- Horizontal -->\r\n<ins class=\"adsbygoogle\"\r\n     style=\"display:block\"\r\n     data-ad-client=\"ca-pub-0127150553352455\"\r\n     data-ad-slot=\"3806776041\"\r\n     data-ad-format=\"auto\"\r\n     data-full-width-responsive=\"true\"><\/ins>\r\n<script>\r\n     (adsbygoogle = window.adsbygoogle || []).push({});\r\n<\/script>\n<\/div>\n<p>S\u00e9zary Syndrome corresponds to 3% of all cutaneous lymphomas, and it is characterized by a triad of manifestations: erythrodermia with pruritus, limphonodomegalia and atypical circulating lymphocytes (referred to as S\u00e9zary or Lutzner cells). Associated clinical manifestations include lagophthalmos, alopecia, palmoplantar hyperkeratosis and onycodystrophy. Erythrodermia may be the progression of previous patches and plaques, developing from idiopathic erythrodermia or emerging de novo.<br \/>\n<!--more--><br \/>\n<strong>Diagnostic Criteria in S\u00e9zary&#8217;s Syndrome (SS)<\/strong><br \/>\nThe diagnostic criteria for the syndrome, as\u00a0recommended by ISCL-EORTC and countersigned in\u00a0the WHO Classification of Tumors of Haematopoietic\u00a0and Lymphoid Tissues, are that the circulating monoclonal\u00a0lymphocyte population should be identified by\u00a0molecular or cytogenetic methods and there should\u00a0be an identity between the circulating T-lymphocyte\u00a0clone and the clone presented in the skin, in addition\u00a0to one of the following: at least 1,000 S\u00e9zary cells per\u00a0mm3 of peripheral blood, an increased population of\u00a0CD4+\/CD7- in peripheral blood with remarkable predominance\u00a0of CD4+ cells in relation to\u00a0CD8+\u00a0(CD4\/CD8 ratio &gt; 10), S\u00e9zary cells with a diameter\u00a0&gt; 14 um representing &gt; 20% of the circulating\u00a0lymphocytes and, some markers like CD2, CD3, CD4\u00a0and CD5 must be absent.<br \/>\nThe staging system used for mycosis fungoides (MF) is also used for\u00a0SS, and by definition the syndrome is classified in\u00a0stage III from the beginning. SS prognosis is poor,\u00a0with a mean survival of 2 to 4 years. Both MF and SS\u00a0patients have an increased chance of developing a\u00a0second malignant neoplasia and even a second lymphoma.\u00a0The opposite also seems to be true, that is,\u00a0patients with a type-B lymphoma may develop type-T\u00a0MF or SS more frequently than the general population.<\/p>\n<p>&nbsp;<\/p>\n<p><strong>References:<\/strong><\/p>\n<ol>\n<li>Willemze R, van Vloten WA, Hermans J, Damsteeg MJ, Meijer CJ. Diagnostic criteria in S\u00e9zary&#8217;s syndrome: a multiparameter study of peripheral blood lymphocytes in 32 patients with erythroderma.\u00a0J Invest Dermatol. 1983 Nov;81(5):392-7.\u00a0<a href=\"https:\/\/pubmed.ncbi.nlm.nih.gov\/6226746\/\" target=\"_blank\" rel=\"noopener noreferrer\">[Medline]<\/a><\/li>\n<li>Hwang ST, Janik JE, Jaffe ES, Wilson WH. Mycosis fungoides and S\u00e9zary syndrome.\u00a0Lancet. 2008 Mar 15;371(9616):945-57.\u00a0<a href=\"https:\/\/pubmed.ncbi.nlm.nih.gov\/18342689\/\" target=\"_blank\" rel=\"noopener noreferrer\">[Medline]<\/a><\/li>\n<li>Vonderheid EC, Bernengo MG. The S\u00e9zary syndrome: hematologic criteria. Hematol Oncol Clin North Am. 2003 Dec;17(6):1367-89,\u00a0<a href=\"https:\/\/pubmed.ncbi.nlm.nih.gov\/14710890\/\" target=\"_blank\" rel=\"noopener noreferrer\">[Medline]<\/a><\/li>\n<li>Yamashita T, Abbade LP, Marques ME, Marques SA. Mycosis fungoides and S\u00e9zary syndrome: clinical, histopathological and immunohistochemical review and update.\u00a0An Bras Dermatol. 2012 Nov-Dec;87(6):817-28\u00a0<a href=\"https:\/\/pubmed.ncbi.nlm.nih.gov\/23197199\/\" target=\"_blank\" rel=\"noopener noreferrer\">[Medline]<\/a><\/li>\n<li>Hristov AC, Tejasvi T, Wilcox RA. Mycosis Fungoides, S\u00e9zary Syndrome, and Cutaneous B-Cell Lymphomas: 2025 Update on Diagnosis, Risk-Stratification, and Management. Am J Hematol. 2025 Sep;100(9):1603-1628. <a href=\"https:\/\/pubmed.ncbi.nlm.nih.gov\/40495407\/\" target=\"_blank\" rel=\"noopener\">[Medline]<\/a><\/li>\n<\/ol>\n<p>&nbsp;<\/p>\n<p>Created Jan 15, 2015.<br \/>\nUp-date Apr 6, 2026.<\/p>\n\n<div style=\"font-size: 0px; height: 0px; line-height: 0px; margin: 0; padding: 0; clear: both;\"><\/div>","protected":false},"excerpt":{"rendered":"<p>Sorry, this entry is only available in Espa\u00f1ol.<\/p>\n","protected":false},"author":2,"featured_media":0,"comment_status":"closed","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"_lmt_disableupdate":"no","_lmt_disable":"no","_exactmetrics_skip_tracking":false,"_exactmetrics_sitenote_active":false,"_exactmetrics_sitenote_note":"","_exactmetrics_sitenote_category":0,"footnotes":""},"categories":[34],"tags":[16,15,14,841,5,842,4],"class_list":["post-2353","post","type-post","status-publish","format-standard","hentry","category-dermatology","tag-criteria","tag-criterios","tag-diagnostic","tag-sezary","tag-sindrome","tag-ss","tag-syndrome"],"modified_by":"Guillermo Firman","_links":{"self":[{"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/posts\/2353","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/users\/2"}],"replies":[{"embeddable":true,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/comments?post=2353"}],"version-history":[{"count":4,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/posts\/2353\/revisions"}],"predecessor-version":[{"id":10732,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/posts\/2353\/revisions\/10732"}],"wp:attachment":[{"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/media?parent=2353"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/categories?post=2353"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/tags?post=2353"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}