{"id":5150,"date":"2025-09-23T22:24:05","date_gmt":"2025-09-23T22:24:05","guid":{"rendered":"https:\/\/atlasemhematologia.com.br\/?p=5150"},"modified":"2025-09-23T22:24:05","modified_gmt":"2025-09-23T22:24:05","slug":"purpura-trombocitopenica-trombotica","status":"publish","type":"post","link":"https:\/\/atlasemhematologia.com.br\/blog\/purpura-trombocitopenica-trombotica\/","title":{"rendered":"P\u00farpura Trombocitop\u00eanica Tromb\u00f3tica (PTT)"},"content":{"rendered":"\n<p class=\"wp-block-paragraph\">Doen\u00e7a que leva a forma\u00e7\u00e3o de trombos nos vasos sangu\u00edneos, caracterizada  pela defici\u00eancia de clivagem do fator de Von Willebrand (FvW), pela enzima conhecida como ADAMTS13, \u00e9 a condi\u00e7\u00e3o chamada P\u00farpura Trombocitop\u00eanica Tromb\u00f3tica (PTT).<\/p>\n\n\n\n<div class=\"wp-block-image\"><figure class=\"aligncenter size-large is-resized\"><img fetchpriority=\"high\" decoding=\"async\" src=\"https:\/\/atlasemhematologia.com.br\/blog\/wp-content\/uploads\/2023\/08\/image-2.png\" alt=\"\" class=\"wp-image-5175\" width=\"453\" height=\"252\"\/><figcaption>L\u00e2mina de paciente com a PTT<\/figcaption><\/figure><\/div>\n\n\n\n<h4 class=\"wp-block-heading\"><strong>Fator de von Willebrand (FvW)<\/strong><\/h4>\n\n\n\n<p class=\"wp-block-paragraph\">C\u00e9lulas endoteliais e megacari\u00f3citos produzem o Fator de Von Willebrand (FvW), uma glicoprote\u00edna que desempenha um papel crucial no processo de coagula\u00e7\u00e3o. A  ADAMTS13 regula o tamanho dos FvW, liberados como mult\u00edmeros (mol\u00e9culas maiores), estes s\u00e3o cortados em mol\u00e9culas menores para que possam desempenhar seu papel pr\u00f3-coagulante.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">A defici\u00eancia da ADAMTS13 impede a correta cliva\u00e7\u00e3o dos mult\u00edmeros, o que torna mais f\u00e1cil a forma\u00e7\u00e3o de trombos na circula\u00e7\u00e3o. Este processo pode desencadear graves complica\u00e7\u00f5es.  Entre eles, a classica forma\u00e7\u00e3o de esquiz\u00f3citos, gerados pelo choque de hem\u00e1cias \u00edntegras, que colidem com os trombos.<\/p>\n\n\n\n<div class=\"wp-block-image\"><figure class=\"aligncenter size-large is-resized\"><img decoding=\"async\" src=\"https:\/\/atlasemhematologia.com.br\/blog\/wp-content\/uploads\/2023\/08\/image.png\" alt=\"\" class=\"wp-image-5172\" width=\"686\" height=\"236\" srcset=\"https:\/\/atlasemhematologia.com.br\/blog\/wp-content\/uploads\/2023\/08\/image.png 1018w, https:\/\/atlasemhematologia.com.br\/blog\/wp-content\/uploads\/2023\/08\/image-300x103.png 300w, https:\/\/atlasemhematologia.com.br\/blog\/wp-content\/uploads\/2023\/08\/image-768x264.png 768w, https:\/\/atlasemhematologia.com.br\/blog\/wp-content\/uploads\/2023\/08\/image-705x242.png 705w\" sizes=\"(max-width: 686px) 100vw, 686px\" \/><figcaption>Ilustra\u00e7\u00e3o que demonstra como a cliva\u00e7\u00e3o inadequada do Fator de von Willebrand (estrutura verde) contribui para aglomerados de trombos. A ADAMTS13 \u00e9 a estrutura roxa.<\/figcaption><\/figure><\/div>\n\n\n\n<h4 class=\"wp-block-heading\">Classifica\u00e7\u00e3o<\/h4>\n\n\n\n<p class=\"wp-block-paragraph\">A PTT se divide em dois tipos: cong\u00eanito e adquirido.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">No tipo cong\u00eanito, \u00e9 associado a uma reduzida atividade da enzima ADAMTS13. Esta redu\u00e7\u00e3o muitas vezes resulta de muta\u00e7\u00f5es gen\u00e9ticas na prote\u00edna e segue um padr\u00e3o de heran\u00e7a autoss\u00f4mica recessiva.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">No tipo adquirido, a PTT \u00e9 subdividida em imunomediada e secund\u00e1ria. Na forma imunomediada, h\u00e1 a presen\u00e7a de autoanticorpos contra a ADAMTS13. Na forma secund\u00e1ria, a condi\u00e7\u00e3o \u00e9 desencadeada pela libera\u00e7\u00e3o excessiva de mult\u00edmeros do FvW por c\u00e9lulas estimuladas, ultrapassando a capacidade de degrada\u00e7\u00e3o da enzima . Nesse cen\u00e1rio, a ADAMTS13 n\u00e3o consegue realizar adequadamente a clivagem dos mult\u00edmeros do FvW, resultando em um excesso.<\/p>\n\n\n\n<h4 class=\"wp-block-heading\"><strong>Caracter\u00edsticas do hemograma<\/strong><\/h4>\n\n\n\n<ul class=\"wp-block-list\"><li>Anemia;<\/li><li>Poiquilocitose, com esquiz\u00f3citos e querat\u00f3citos mais frequentemente; <\/li><li>Policromasia;<\/li><li>Eritroblastos est\u00e3o presentes;<\/li><li>A contagem de leuc\u00f3citos geralmente se mant\u00e9m dentro das faixas normais;<\/li><li>Trombocitopenia \u00e9 identificada. <\/li><li>Macroplaquetas e plaquetas gigantes \u00e9 comum.<\/li><\/ul>\n\n\n\n<div class=\"wp-block-image\"><figure class=\"aligncenter size-large is-resized\"><img decoding=\"async\" src=\"https:\/\/atlasemhematologia.com.br\/blog\/wp-content\/uploads\/2023\/08\/image-1.png\" alt=\"\" class=\"wp-image-5174\" width=\"527\" height=\"338\" srcset=\"https:\/\/atlasemhematologia.com.br\/blog\/wp-content\/uploads\/2023\/08\/image-1.png 733w, https:\/\/atlasemhematologia.com.br\/blog\/wp-content\/uploads\/2023\/08\/image-1-300x193.png 300w, https:\/\/atlasemhematologia.com.br\/blog\/wp-content\/uploads\/2023\/08\/image-1-705x453.png 705w\" sizes=\"(max-width: 527px) 100vw, 527px\" \/><figcaption>Hemograma real de um paciente com PTT<\/figcaption><\/figure><\/div>\n\n\n\n<p class=\"wp-block-paragraph\">A PTT apresenta uma variedade de sintomas, frequentemente incluindo fadiga, fraqueza, febre, dores de cabe\u00e7a, confus\u00e3o mental, complica\u00e7\u00f5es card\u00edacas e renais, isso devido \u00e0 m\u00e1 circula\u00e7\u00e3o sangu\u00ednea. Os m\u00e9dicos diagnosticam a doen\u00e7a atrav\u00e9s da avalia\u00e7\u00e3o cl\u00ednica e de testes laboratoriais complementares, como a medi\u00e7\u00e3o da pr\u00f3pria ADAMTS13 e de anticorpos contra ela.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">Os m\u00e9dicos geralmente tratam a condi\u00e7\u00e3o em um ambiente hospitalar, utilizando terapias de reposi\u00e7\u00e3o de plasma para administrar a enzima ADAMTS13 ausente ou deficiente, al\u00e9m de realizar trocas de plasma para eliminar os autoanticorpos. Em situa\u00e7\u00f5es mais graves, terapias de suporte, como a di\u00e1lise, podem ser necess\u00e1rias para tratar a insufici\u00eancia renal.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">A compreens\u00e3o da fisiopatologia das doen\u00e7as \u00e9 crucial para identificar as causas das altera\u00e7\u00f5es no hemograma e avaliar a gravidade patol\u00f3gica de cada condi\u00e7\u00e3o.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\"><strong>Quer se tornar um mestre na hematologia? Clique no bot\u00e3o e embarque nessa jornada de aprendizado: <\/strong><a href=\"https:\/\/hematologianapratica.com.br\/?hsrc=YmFubmVyc2Jsb2c%3D\">Hematologia Na Pr\u00e1tica \u2013 Seu portal de conte\u00fados<\/a><\/p>\n\n\n\n<h4 class=\"wp-block-heading\">Refer\u00eancias<\/h4>\n\n\n\n<ol class=\"wp-block-list\"><li>NAOUM, Fl\u00e1vio Augusto. Doen\u00e7as que alteram os exames hematol\u00f3gicos. 2\u00aa edi\u00e7\u00e3o. Editora Atheneu, 2017.<\/li><li>OLIVEIRA, Raimundo Ant\u00f4nio Gomes. Hemograma: como fazer e interpretar. 1\u00aa edi\u00e7\u00e3o. Editora LMP, 2007.<\/li><li>BAIN, Barbara J. C\u00e9lulas sangu\u00edneas &#8211; um guia pr\u00e1tico. 5\u00aa edi\u00e7\u00e3o. Editora Artmed, 2016.<\/li><li>Campos, Lucia M. A. et al. P\u00farpura trombocitop\u00eanica tromb\u00f3tica na apresenta\u00e7\u00e3o de pacientes com l\u00fapus eritematoso sist\u00eamico juvenil. Revista Brasileira de Reumatologia. 2013, v. 53, n. 1, pp. 123-126. Dispon\u00edvel em: Epub 09&nbsp;Abr&nbsp;2013. ISSN 1809-4570. (Link para usar no blog: <a href=\"https:\/\/www.scielo.br\/j\/rbr\/a\/ZqTDtqrS8ZJHJqW6GJnWn4v\/#ModalArticles\">https:\/\/www.scielo.br\/j\/rbr\/a\/ZqTDtqrS8ZJHJqW6GJnWn4v\/#ModalArticles<\/a>)<\/li><\/ol>\n","protected":false},"excerpt":{"rendered":"<p>Doen\u00e7a que leva a forma\u00e7\u00e3o de trombos nos vasos sangu\u00edneos, caracterizada pela defici\u00eancia de clivagem do fator de Von Willebrand (FvW), pela enzima conhecida como ADAMTS13, \u00e9 a condi\u00e7\u00e3o chamada P\u00farpura Trombocitop\u00eanica Tromb\u00f3tica (PTT). Fator de von Willebrand (FvW) C\u00e9lulas endoteliais e megacari\u00f3citos produzem o Fator de Von Willebrand (FvW), uma glicoprote\u00edna que desempenha um [&hellip;]<\/p>\n","protected":false},"author":3,"featured_media":5214,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"pagelayer_contact_templates":[],"_pagelayer_content":"","footnotes":""},"categories":[5],"tags":[],"class_list":["post-5150","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-sem-categoria"],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v27.2 - https:\/\/yoast.com\/product\/yoast-seo-wordpress\/ -->\n<title>P\u00farpura Trombocitop\u00eanica Tromb\u00f3tica (PTT) - Blog - Atlas em Hematologia<\/title>\n<meta name=\"description\" content=\"- fator de Von Willebrand pela enzima conhecida como ADAMTS13, \u00e9 o que caracteriza a condi\u00e7\u00e3o chamada P\u00farpura Trombocitop\u00eanica Tromb\u00f3tica.\" \/>\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\/purpura-trombocitopenica-trombotica\/\" \/>\n<meta property=\"og:locale\" content=\"pt_BR\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"P\u00farpura Trombocitop\u00eanica Tromb\u00f3tica (PTT) - 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