{"id":17523,"date":"2026-05-03T12:29:15","date_gmt":"2026-05-03T12:29:15","guid":{"rendered":"https:\/\/atlasemhematologia.com.br\/blog\/?p=17523"},"modified":"2026-04-30T12:31:22","modified_gmt":"2026-04-30T12:31:22","slug":"sindrome-de-sezary","status":"publish","type":"post","link":"https:\/\/atlasemhematologia.com.br\/blog\/sindrome-de-sezary\/","title":{"rendered":"S\u00edndrome de S\u00e9zary"},"content":{"rendered":"\n<p>A s\u00edndrome de S\u00e9zary (SS) \u00e9 um linfoma cut\u00e2neo eritrod\u00e9rmico de c\u00e9lulas T maduras, caracterizado pela presen\u00e7a de linf\u00f3citos T CD4+ neopl\u00e1sicos com componente leuc\u00eamico. Trata-se de uma forma agressiva de linfoma cut\u00e2neo, associada \u00e0 eritrodermia generalizada, linfadenopatia e circula\u00e7\u00e3o de c\u00e9lulas neopl\u00e1sicas no sangue perif\u00e9rico, denominadas c\u00e9lulas de S\u00e9zary.<\/p>\n\n\n\n<p>Nesse contexto, a atua\u00e7\u00e3o do laborat\u00f3rio \u00e9 essencial, especialmente na identifica\u00e7\u00e3o morfol\u00f3gica dessas c\u00e9lulas e na confirma\u00e7\u00e3o diagn\u00f3stica por meio da imunofenotipagem. A integra\u00e7\u00e3o entre avalia\u00e7\u00e3o cl\u00ednica, hematol\u00f3gica e citol\u00f3gica permite estabelecer o diagn\u00f3stico e determinar a extens\u00e3o da doen\u00e7a.<\/p>\n\n\n\n<p><\/p>\n\n\n\n<figure class=\"wp-block-image size-full\"><img fetchpriority=\"high\" decoding=\"async\" width=\"504\" height=\"416\" src=\"https:\/\/atlasemhematologia.com.br\/blog\/wp-content\/uploads\/2026\/04\/image-13.png\" alt=\"\" class=\"wp-image-17525\" srcset=\"https:\/\/atlasemhematologia.com.br\/blog\/wp-content\/uploads\/2026\/04\/image-13.png 504w, https:\/\/atlasemhematologia.com.br\/blog\/wp-content\/uploads\/2026\/04\/image-13-300x248.png 300w\" sizes=\"(max-width: 504px) 100vw, 504px\" \/><\/figure>\n\n\n\n<p>Caso de S\u00e9zary atendido no Time Atlas.<\/p>\n\n\n\n<h3 class=\"wp-block-heading\"><strong>1. Aspectos cl\u00ednicos e fisiopatol\u00f3gicos da s\u00edndrome de S\u00e9zary<\/strong><\/h3>\n\n\n\n<p>A s\u00edndrome de S\u00e9zary integra o grupo dos linfomas cut\u00e2neos de c\u00e9lulas T e apresenta caracter\u00edsticas cl\u00ednicas marcantes, sendo a eritrodermia generalizada um dos principais achados. A eritrodermia pode ser definida como uma erup\u00e7\u00e3o cut\u00e2nea intensa, disseminada, pruriginosa e esfoliativa, que pode representar o surgimento de novas les\u00f5es ou a progress\u00e3o de altera\u00e7\u00f5es cut\u00e2neas pr\u00e9-existentes.<\/p>\n\n\n\n<p>A doen\u00e7a origina-se da prolifera\u00e7\u00e3o monoclonal de linf\u00f3citos T p\u00f3s-t\u00edmicos, geralmente com fen\u00f3tipo helper, que apresentam elevada afinidade pela pele. Embora a etiologia exata permane\u00e7a desconhecida, h\u00e1 evid\u00eancias que sugerem a participa\u00e7\u00e3o de fatores gen\u00e9ticos, ambientais e infecciosos no desenvolvimento da s\u00edndrome.<\/p>\n\n\n\n<p>Al\u00e9m das manifesta\u00e7\u00f5es cut\u00e2neas, altera\u00e7\u00f5es extra cut\u00e2neas s\u00e3o frequentes e podem incluir:<\/p>\n\n\n\n<ul class=\"wp-block-list\">\n<li>Linfadenopatia<\/li>\n\n\n\n<li>Hepatoesplenomegalia<\/li>\n\n\n\n<li>Infiltrados pulmonares ou derrame pleural<\/li>\n\n\n\n<li>Comprometimento sist\u00eamico progressivo<\/li>\n<\/ul>\n\n\n\n<p>Assim, o progn\u00f3stico da doen\u00e7a est\u00e1 diretamente relacionado \u00e0 extens\u00e3o do envolvimento cut\u00e2neo, hematol\u00f3gico e visceral no momento do diagn\u00f3stico.<\/p>\n\n\n\n<hr class=\"wp-block-separator has-alpha-channel-opacity\"\/>\n\n\n\n<h3 class=\"wp-block-heading\"><strong>2. Caracter\u00edsticas morfol\u00f3gicas das c\u00e9lulas de S\u00e9zary<\/strong><\/h3>\n\n\n\n<p>A an\u00e1lise microsc\u00f3pica do sangue perif\u00e9rico desempenha papel fundamental na suspeita diagn\u00f3stica da s\u00edndrome de S\u00e9zary. As c\u00e9lulas de S\u00e9zary apresentam morfologia bastante caracter\u00edstica, sendo descritas como linf\u00f3citos an\u00f4malos de m\u00e9dio a grande porte, com citoplasma abundante e basof\u00edlico.<\/p>\n\n\n\n<p>O n\u00facleo dessas c\u00e9lulas \u00e9 altamente irregular e convoluto, frequentemente descrito como <strong>cerebriforme<\/strong>, devido ao seu aspecto semelhante \u00e0s circunvolu\u00e7\u00f5es cerebrais. A cromatina nuclear encontra-se condensada, e os nucl\u00e9olos geralmente n\u00e3o s\u00e3o vis\u00edveis.<\/p>\n\n\n\n<p>Em est\u00e1gios mais avan\u00e7ados da doen\u00e7a, pode ocorrer infiltra\u00e7\u00e3o cut\u00e2nea significativa, caracterizada por fen\u00f4menos como:<\/p>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Epidermotropismo<\/strong>, que corresponde \u00e0 infiltra\u00e7\u00e3o da epiderme por linf\u00f3citos tumorais<\/li>\n\n\n\n<li><strong>Microabscessos de Pautrier<\/strong>, formados por agregados de c\u00e9lulas neopl\u00e1sicas na epiderme<\/li>\n<\/ul>\n\n\n\n<p>Portanto, a revis\u00e3o microsc\u00f3pica cuidadosa constitui etapa essencial para a identifica\u00e7\u00e3o dessas altera\u00e7\u00f5es morfol\u00f3gicas e para o acompanhamento evolutivo da doen\u00e7a.<\/p>\n\n\n\n<hr class=\"wp-block-separator has-alpha-channel-opacity\"\/>\n\n\n\n<h3 class=\"wp-block-heading\"><strong>3. Imunofenotipagem e confirma\u00e7\u00e3o diagn\u00f3stica<\/strong><\/h3>\n\n\n\n<p>A imunofenotipagem por citometria de fluxo \u00e9 uma ferramenta indispens\u00e1vel na confirma\u00e7\u00e3o diagn\u00f3stica da s\u00edndrome de S\u00e9zary, permitindo identificar o perfil imunol\u00f3gico das c\u00e9lulas neopl\u00e1sicas e distinguir essa condi\u00e7\u00e3o de outras doen\u00e7as linfoproliferativas.<\/p>\n\n\n\n<p>As c\u00e9lulas de S\u00e9zary apresentam tipicamente express\u00e3o de marcadores de c\u00e9lulas T maduras, como:<\/p>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>CD3 positivo<\/strong><\/li>\n\n\n\n<li><strong>CD4 positivo<\/strong><\/li>\n<\/ul>\n\n\n\n<p>Al\u00e9m disso, observa-se frequentemente perda de express\u00e3o de alguns ant\u00edgenos de c\u00e9lulas T maduras, como:<\/p>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>CD2<\/strong><\/li>\n\n\n\n<li><strong>CD5<\/strong><\/li>\n\n\n\n<li><strong>CD7<\/strong><\/li>\n<\/ul>\n\n\n\n<p>Essas altera\u00e7\u00f5es imunofenot\u00edpicas s\u00e3o consideradas altamente sugestivas da doen\u00e7a e auxiliam tanto no diagn\u00f3stico quanto no monitoramento da carga tumoral ao longo do tratamento.<\/p>\n\n\n\n<p><\/p>\n\n\n\n<figure class=\"wp-block-image size-large\"><a href=\"https:\/\/eventos.atlasemhematologia.com.br\/listadeesperatimeblogatlas\"><img decoding=\"async\" width=\"1030\" height=\"276\" src=\"https:\/\/atlasemhematologia.com.br\/blog\/wp-content\/uploads\/2026\/03\/BANNER-BLOG-ATLAS-1030x276.png\" alt=\"\" class=\"wp-image-17314\" srcset=\"https:\/\/atlasemhematologia.com.br\/blog\/wp-content\/uploads\/2026\/03\/BANNER-BLOG-ATLAS-1030x276.png 1030w, https:\/\/atlasemhematologia.com.br\/blog\/wp-content\/uploads\/2026\/03\/BANNER-BLOG-ATLAS-300x80.png 300w, https:\/\/atlasemhematologia.com.br\/blog\/wp-content\/uploads\/2026\/03\/BANNER-BLOG-ATLAS-768x206.png 768w, https:\/\/atlasemhematologia.com.br\/blog\/wp-content\/uploads\/2026\/03\/BANNER-BLOG-ATLAS-1536x411.png 1536w, https:\/\/atlasemhematologia.com.br\/blog\/wp-content\/uploads\/2026\/03\/BANNER-BLOG-ATLAS-2048x549.png 2048w\" sizes=\"(max-width: 1030px) 100vw, 1030px\" \/><\/a><\/figure>\n\n\n\n<hr class=\"wp-block-separator has-alpha-channel-opacity\"\/>\n\n\n\n<p><strong>Refer\u00eancias:<\/strong><\/p>\n\n\n\n<p>OLIVEIRA, Larissa Santana; ALCANTARA, Thiago Ruan de Lima. <em>Atlas em hematologia: um guia visual para a identifica\u00e7\u00e3o de c\u00e9lulas sangu\u00edneas<\/em>. 1. ed. Salvador: Oxente, 2025.<\/p>\n\n\n\n<p>Atlas de hematologia: cl\u00ednica hematol\u00f3gica ilustrada\/ Therezinha Ferreira Lorenzi, coordenadora. &#8211; Rio de Janeiro: Guanabara Koogan, 2006<\/p>\n\n\n\n<p>Miyashiro D, Souza BCE, Torrealba MP, Manfrere KCG, Sato MN, Sanches JA. O papel do microambiente tumoral na patog\u00eanese da s\u00edndrome de S\u00e9zary. 2022 PMID: 35055124<\/p>\n\n\n\n<p>WEINSTOCK, Martin A.; HORM, John W. Micose fungoide nos Estados Unidos: incid\u00eancia crescente e epidemiologia descritiva. <strong>Jama<\/strong>, v. 1, p\u00e1g. 42-46, 1988.<\/p>\n\n\n\n<p>KUZEL,TM; ROENIGK JR, HH; ROSEN, ST Micose fungoide e s\u00edndrome de S\u00e9zary: uma revis\u00e3o da patog\u00eanese, diagn\u00f3stico e terapia. <strong>Revista de oncologia cl\u00ednica<\/strong>, v. 9, n. 7, p\u00e1g. 1298-1313, 1991.<\/p>\n","protected":false},"excerpt":{"rendered":"<p>A s\u00edndrome de S\u00e9zary (SS) \u00e9 um linfoma cut\u00e2neo eritrod\u00e9rmico de c\u00e9lulas T maduras, caracterizado pela presen\u00e7a de linf\u00f3citos T CD4+ neopl\u00e1sicos com componente leuc\u00eamico. Trata-se de uma forma agressiva de linfoma cut\u00e2neo, associada \u00e0 eritrodermia generalizada, linfadenopatia e circula\u00e7\u00e3o de c\u00e9lulas neopl\u00e1sicas no sangue perif\u00e9rico, denominadas c\u00e9lulas de S\u00e9zary. Nesse contexto, a atua\u00e7\u00e3o do [&hellip;]<\/p>\n","protected":false},"author":6,"featured_media":17526,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"pagelayer_contact_templates":[],"_pagelayer_content":"","footnotes":""},"categories":[18],"tags":[],"class_list":["post-17523","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-doencas-hematologicas"],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v27.2 - https:\/\/yoast.com\/product\/yoast-seo-wordpress\/ -->\n<title>S\u00edndrome de S\u00e9zary - Blog - Atlas em Hematologia<\/title>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/atlasemhematologia.com.br\/blog\/sindrome-de-sezary\/\" \/>\n<meta property=\"og:locale\" content=\"pt_BR\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"S\u00edndrome de S\u00e9zary - Blog - Atlas em Hematologia\" \/>\n<meta property=\"og:description\" content=\"A s\u00edndrome de S\u00e9zary (SS) \u00e9 um linfoma cut\u00e2neo eritrod\u00e9rmico de c\u00e9lulas T maduras, caracterizado pela presen\u00e7a de linf\u00f3citos T CD4+ neopl\u00e1sicos com componente leuc\u00eamico. Trata-se de uma forma agressiva de linfoma cut\u00e2neo, associada \u00e0 eritrodermia generalizada, linfadenopatia e circula\u00e7\u00e3o de c\u00e9lulas neopl\u00e1sicas no sangue perif\u00e9rico, denominadas c\u00e9lulas de S\u00e9zary. 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