{"id":2466,"date":"2009-10-11T23:33:44","date_gmt":"2009-10-11T23:33:44","guid":{"rendered":"https:\/\/medicalcriteria.com\/web\/hemalps\/"},"modified":"2025-05-14T19:08:59","modified_gmt":"2025-05-14T19:08:59","slug":"hemalps","status":"publish","type":"post","link":"https:\/\/medicalcriteria.com\/web\/es\/hemalps\/","title":{"rendered":"Criterios Diagn\u00f3stico para S\u00edndrome Linfoproliferativo Autoinmune"},"content":{"rendered":"<div class=\"7ca9444b8380138fe077da07353677a4\" 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>El s\u00edndrome linfoproliferativo autoinmune (ALPS) se caracteriza por la desregulaci\u00f3n del sistema inmune debido a la incapacidad de regular la homeostasis de los linfocitos mediante el proceso de apoptosis de los linfocitos (una forma de muerte celular programada). Las consecuencias de esto incluyen la enfermedad linfoproliferativa, manifestada por linfadenopat\u00eda, hepatomegalia, esplenomegalia y un mayor riesgo de linfoma, as\u00ed como una enfermedad autoinmune, que generalmente involucra c\u00e9lulas sangu\u00edneas.<\/p>\n<p><!--more--><\/p>\n<p><strong>Criterios Diagn\u00f3stico para S\u00edndrome Linfoproliferativa Autoinmune<\/strong><\/p>\n<p><strong>Requerido<\/strong><\/p>\n<ol>\n<li>Linfoproliferaci\u00f3n cr\u00f3nica no maligna<\/li>\n<li>Defectos in vitro de apoptosis de linfocitos Fas mediada<\/li>\n<li>Al menos el 1% TCR (alfa)\/(beta) + CD3 +, CD4 y CD8, c\u00e9lulas (DNTs) en sangre perif\u00e9rica o tejido linfoide<\/li>\n<\/ol>\n<p><strong>Soporte<\/strong><\/p>\n<ol>\n<li>Anticuerpos autoinmunes<\/li>\n<li>Mutaciones en el gen Fas, gen Fas ligando, o genes de proteasas que median la apoptosis 8 o 10<\/li>\n<\/ol>\n<p>Abreviaturas: TCR, receptor de c\u00e9lulas T; DNTs, c\u00e9lulas T doble negativas<\/p>\n<p>&nbsp;<\/p>\n<p><strong>Criterios diagn\u00f3stico revisados \u200b\u200bpara ALPS<\/strong><\/p>\n<p><strong>Requerido<\/strong><\/p>\n<ol>\n<li>Linfadenopat\u00eda o esplenomegalia cr\u00f3nica (&gt; 6 meses), no maligna, no infecciosa o ambas<\/li>\n<li>C\u00e9lulas CD3 + TCR\u03b1\u03b2 + CD4\u2212 CD8\u2212 DNT elevadas (\u2265 1.5% de linfocitos totales o 2.5% de linfocitos CD3 +) en el contexto de conteos de linfocitos normales o elevados<\/li>\n<\/ol>\n<p><strong>Accesorio<\/strong><\/p>\n<p>Primario<\/p>\n<ol>\n<li>Apoptosis linfoc\u00edtica defectuosa (en 2 pruebas separadas)<\/li>\n<li>Mutaci\u00f3n patog\u00e9nica som\u00e1tica o de la l\u00ednea germinal en FAS, FASLG o CASP10<\/li>\n<\/ol>\n<p>Secundario<\/p>\n<ol>\n<li>Niveles elevados de sFASL en plasma (&gt;200 pg\/ml) O niveles elevados de interleucina-10 en plasma (&gt;20 pg\/ml) O niveles elevados de vitamina B12 en suero o plasma (&gt;1500 ng\/L) O niveles elevados de interleucina-18 en plasma &gt;500 pg\/mL<\/li>\n<li>Hallazgos inmunohistol\u00f3gicos t\u00edpicos revisados \u200b\u200bpor un hematopat\u00f3logo experimentado<\/li>\n<li>Citopenias autoinmunes (anemia hemol\u00edtica, trombocitopenia o neutropenia) Y niveles elevados de inmunoglobulina G (hipergammaglobulinemia policlonal)<\/li>\n<li>Antecedentes familiares de una linfoproliferaci\u00f3n no maligna\/no infecciosa con o sin autoinmunidad<\/li>\n<\/ol>\n<p>Un diagn\u00f3stico definitivo se basa en la presencia de ambos criterios requeridos m\u00e1s un criterio accesorio primario. Un diagn\u00f3stico probable se basa en la presencia de ambos criterios requeridos m\u00e1s un criterio accesorio secundario.<\/p>\n<p>&nbsp;<\/p>\n<p><strong>Bibliograf\u00eda:<\/strong><\/p>\n<ol>\n<li>Teachey DT, Manno CS, Axsom KM, Andrews T, Choi JK, Greenbaum BH, McMann JM, Sullivan KE, Travis SF, Grupp SA. Unmasking Evans syndrome: T-cell phenotype and apoptotic response reveal autoimmune lymphoproliferative syndrome (ALPS). Blood. 2005 Mar 15;105(6):2443-8. <a href=\"https:\/\/pubmed.ncbi.nlm.nih.gov\/15542578\/\" target=\"_blank\" rel=\"noopener noreferrer\">[Medline]<\/a><\/li>\n<li>Bleesing JJ, Straus SE, Fleisher TA. Autoimmune lymphoproliferative syndrome: a human disorder of abnormal lymphocyte survival. Pediatr Clin North Am. 2000;47: 1291-1310. <a href=\"https:\/\/pubmed.ncbi.nlm.nih.gov\/11130997\/\" target=\"_blank\" rel=\"noopener noreferrer\">[Medline]<\/a><\/li>\n<li>Oliveira JB, Bleesing JJ, Dianzani U, et al. Revised diagnostic criteria and classification for the autoimmune lymphoproliferative syndrome (ALPS): report from the 2009 NIH International Workshop. Blood. 2010;116(14):e35\u2013e40. <a href=\"https:\/\/pubmed.ncbi.nlm.nih.gov\/20538792\/\" target=\"_blank\" rel=\"noopener noreferrer\">[Medline]<\/a><\/li>\n<\/ol>\n<p>&nbsp;<\/p>\n<p>Creado: Oct 20, 2009<br \/>\nActualizado Feb 05, 2020.<\/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>El s\u00edndrome linfoproliferativo autoinmune (ALPS) se caracteriza por la desregulaci\u00f3n del sistema inmune debido a la incapacidad de regular la homeostasis de los linfocitos mediante el proceso de apoptosis de los linfocitos (una forma de muerte celular programada). Las consecuencias de esto incluyen la enfermedad linfoproliferativa, manifestada por linfadenopat\u00eda, hepatomegalia, esplenomegalia y un mayor riesgo [&hellip;]<\/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":[171],"tags":[1385,250,251,16,15,14,13,1381,1384,1383,5,4],"class_list":["post-2466","post","type-post","status-publish","format-standard","hentry","category-hematology","tag-alps","tag-autoimmune","tag-autoinmune","tag-criteria","tag-criterios","tag-diagnostic","tag-diagnostico","tag-hematology","tag-linfoproliferativo","tag-lymphoproliferative","tag-sindrome","tag-syndrome"],"modified_by":"Guillermo Firman","_links":{"self":[{"href":"https:\/\/medicalcriteria.com\/web\/es\/wp-json\/wp\/v2\/posts\/2466","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/medicalcriteria.com\/web\/es\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/medicalcriteria.com\/web\/es\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/medicalcriteria.com\/web\/es\/wp-json\/wp\/v2\/users\/2"}],"replies":[{"embeddable":true,"href":"https:\/\/medicalcriteria.com\/web\/es\/wp-json\/wp\/v2\/comments?post=2466"}],"version-history":[{"count":4,"href":"https:\/\/medicalcriteria.com\/web\/es\/wp-json\/wp\/v2\/posts\/2466\/revisions"}],"predecessor-version":[{"id":9048,"href":"https:\/\/medicalcriteria.com\/web\/es\/wp-json\/wp\/v2\/posts\/2466\/revisions\/9048"}],"wp:attachment":[{"href":"https:\/\/medicalcriteria.com\/web\/es\/wp-json\/wp\/v2\/media?parent=2466"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/medicalcriteria.com\/web\/es\/wp-json\/wp\/v2\/categories?post=2466"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/medicalcriteria.com\/web\/es\/wp-json\/wp\/v2\/tags?post=2466"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}