{"id":4544,"date":"2020-11-11T13:03:12","date_gmt":"2020-11-11T13:03:12","guid":{"rendered":"https:\/\/dermatly.com\/sindrome-de-chediak-higashi\/"},"modified":"2026-03-10T10:10:37","modified_gmt":"2026-03-10T10:10:37","slug":"chediak-higashi-syndrome","status":"publish","type":"glossary","link":"https:\/\/dermatly.com\/en\/sindrome-de-chediak-higashi\/","title":{"rendered":"Chediak-Higashi Syndrome"},"content":{"rendered":"<section class=\"textBlock\">\n<h2>\u00bfQu\u00e9 es Chediak-Higashi? <span class=\"term\" data-term-id=\"350\">s\u00edndrome<\/span>?<\/h2>\n<p class=\"normal\">El s\u00edndrome de Chediak-Higashi es un trastorno infantil que interfiere con la funci\u00f3n del sistema inmunol\u00f3gico.  Tambi\u00e9n se conoce como s\u00edndrome de Begnez-Cesar, <span class=\"term\" data-term-id=\"438\">leucocito<\/span> anomal\u00eda albinismo o defecto en asesino natural <span class=\"term\" data-term-id=\"421\">linfocitos<\/span>.<\/p>\n<p class=\"normal\">Fue descrito por primera vez en 1943 por el Dr. Beguez-Cesar. <\/p>\n<h2>\u00bfQui\u00e9n contrae el s\u00edndrome de Chediak-Higashi?<\/h2>\n<p class=\"normal\">El s\u00edndrome de Chediak-Higashi es poco com\u00fan.<\/p>\n<ul>\n<li>Se ha informado de unos 200 a 500 casos en todo el mundo.<\/li>\n<li>los <span class=\"term\" data-term-id=\"578\">incidencia<\/span> es igual en hombres y mujeres.<\/li>\n<li>Afecta a todas las razas, pero los investigadores sospechan que su ocurrencia puede estar subestimada.<\/li>\n<\/ul>\n<div id=\"square-placement-country-holder\" class=\"country-dependent advert\">\n<div class=\"box-placement square-placement\">\n<div class=\"advert__frame\">\n                        <!-- \/340621630\/dermnet_mobile_afterfirstsection_300x250 --><\/p>\n<div style=\"display: none;\" id=\"hidden-div-gpt-ad-1484122037505-8\" class=\"[ js-hideable ]\"><\/div>\n<p><script>\n    if (ads_screenwidth < 780 ) {\n        var inline = document.createElement(\"div\");\n        var ad = document.createElement(\"div\");\n\n        ad.id = 'div-gpt-ad-1484122037505-8';\n        ad.style.height=\"250px\";\n        ad.style.width=\"300px;\";\n\n        inline.appendChild(ad);\n        inline.className = \"inline\";\n        var placeholder = document.getElementById(\"hidden-div-gpt-ad-1484122037505-8\");\n        placeholder.parentNode.replaceChild(inline, placeholder);\n        (function () {\n            googletag.cmd.push(function () {\n                googletag.display('div-gpt-ad-1484122037505-8');\n            });\n        })();\n    }\n<\/script>\n                    <\/div>\n<\/p><\/div>\n<\/p><\/div>\n<h2>\u00bfQu\u00e9 causa el s\u00edndrome de Chediak-Higashi?<\/h2>\n<p class=\"normal\">El s\u00edndrome de Chediak-Higashi es un <span class=\"term\" data-term-id=\"828\">autos\u00f3mico<\/span> condici\u00f3n recesiva causada por un <span class=\"term\" data-term-id=\"870\">mutaci\u00f3n<\/span> sobre el <em>LYST<\/em> <span class=\"term\" data-term-id=\"774\">gene<\/span>.  A menudo se debe a los padres <span class=\"term\" data-term-id=\"835\">consanguinidad<\/span>.  los <em>LYST<\/em> El gen afecta la creaci\u00f3n y el mantenimiento del almacenamiento. <span class=\"term\" data-term-id=\"520\">gr\u00e1nulos<\/span> y causa problemas con el transporte de un <span class=\"term\" data-term-id=\"2224\">prote\u00edna<\/span> a trav\u00e9s de las c\u00e9lulas.<\/p>\n<section class=\"textBlock imageLinkBlockTitle\">\n<h5 class=\"colour--primary\">Gen\u00e9tica del s\u00edndrome de Chediak-Higashi *<\/h5>\n<\/section>\n<section class=\"imageLinkBlock\">\n<div class=\"flex\">\n<div class=\"imageLinkBlock__item__image\">\n<p>                        <img decoding=\"async\" src=\"https:\/\/dermatly.com\/wp-content\/uploads\/2020\/11\/Chediak-Higashi-Syndrome__ProtectWyJQcm90ZWN0Il0_FocusFillWzI5NCwyMjIsIngiLDFd.png\" alt=\"S\u00edndrome de Chediak Higashi\"><\/div>\n<p class=\"p-small\">S\u00edndrome de Chediak Higashi<\/p>\n<\/p><\/div>\n<\/section>\n<div class=\"clearfix imageLinkBlock\"><\/div>\n<p style=\"font-size: 10px;\">* Imagen cortes\u00eda de Genetics 4 Medics<\/p>\n<h2>\u00bfCu\u00e1les son los s\u00edntomas cl\u00ednicos del s\u00edndrome de Chediak-Higashi?<\/h2>\n<p class=\"normalCxSpFirst\">Los signos y s\u00edntomas comienzan a afectar a los ni\u00f1os con s\u00edndrome de Chediak-Higashi poco despu\u00e9s del nacimiento o a m\u00e1s tardar a los cinco a\u00f1os.<\/p>\n<ul>\n<li>Albinismo oculocut\u00e1neo, o <span class=\"term\" data-term-id=\"139\">hipopigmentaci\u00f3n<\/span> del <span class=\"term\" data-term-id=\"122\">cabello<\/span>, ojos y piel;  el cabello se describe como de color claro con un brillo met\u00e1lico<\/li>\n<li>Sangrado prolongado y f\u00e1cil formaci\u00f3n de hematomas debido a <span class=\"term\" data-term-id=\"1089\">plaqueta<\/span> <span class=\"term\" data-term-id=\"1935\">anormalidades<\/span><\/li>\n<li>Virales frecuentes, <span class=\"term\" data-term-id=\"937\">bacteriano<\/span> y hongos <span class=\"term\" data-term-id=\"2101\">infecciones<\/span><\/li>\n<li><span class=\"term\" data-term-id=\"706\">Fotosensibilidad<\/span><\/li>\n<li>Nistagmo: movimientos oculares anormales e involuntarios.<\/li>\n<li>Entumecimiento \/ hormigueo en las extremidades debido a <span class=\"term\" data-term-id=\"1374\">perif\u00e9rico<\/span> <span class=\"term\" data-term-id=\"400\">neuropat\u00eda<\/span><\/li>\n<li>Pobre inmunidad debido a una funci\u00f3n y forma anormales de <span class=\"term\" data-term-id=\"424\">c\u00e9lulas asesinas naturales<\/span><\/li>\n<\/ul>\n<p class=\"normal\">El inicio del s\u00edndrome de Chediak-Higashi en la vida adulta se asocia con s\u00edntomas m\u00e1s leves.  Estos pueden incluir:<\/p>\n<ul>\n<li><span class=\"term\" data-term-id=\"732\">Ictericia<\/span><\/li>\n<li>Infecciones de la piel<\/li>\n<li><span class=\"term\" data-term-id=\"805\">Recurrente<\/span> infecciones del <span class=\"term\" data-term-id=\"2260\">senos nasales<\/span> o tracto respiratorio<\/li>\n<li>Engrandecido <span class=\"term\" data-term-id=\"2123\">linfa<\/span> <span class=\"term\" data-term-id=\"2389\">gl\u00e1ndulas<\/span><\/li>\n<li>Enfermedad de las enc\u00edas (gingivitis)<\/li>\n<li><span class=\"term\" data-term-id=\"1928\">Hiperhidrosis<\/span> y miliaria<\/li>\n<li><span class=\"term\" data-term-id=\"1176\">Fiebre<\/span> sin <span class=\"term\" data-term-id=\"145\">infecci\u00f3n<\/span>.<\/li>\n<\/ul>\n<h2 class=\"normal\">Complicaciones del s\u00edndrome de Chediak-Higashi<\/h2>\n<p class=\"normal\">La mayor\u00eda de los pacientes (alrededor del 80%) se someter\u00e1n a una fase acelerada del s\u00edndrome de Chediak-Higashi.  Esta fase est\u00e1 marcada por la reproducci\u00f3n no maligna de gl\u00f3bulos blancos en m\u00faltiples \u00f3rganos del cuerpo.  Esta fase acelerada puede ser <span class=\"term\" data-term-id=\"1497\">precipitado<\/span> por la presencia de una infecci\u00f3n viral, y a menudo es fatal si ocurre en la infancia.  Esta fase se denomina linfohistiocitosis hemofagoc\u00edtica (HLH).<\/p>\n<div id=\"square-placement-country-holder\" class=\"country-dependent advert\">\n<div class=\"box-placement square-placement\">\n<div class=\"advert__frame\">\n                        <!-- \/340621630\/dermnet_mobile_after4sections_300x250' --><\/p>\n<div style=\"display: none;\" id=\"hidden-div-gpt-ad-1484122037505-9\" class=\"[ js-hideable ]\"><\/div>\n<p><script>\n    if (ads_screenwidth < 780 ) {\n        var inline = document.createElement(\"div\");\n        var ad = document.createElement(\"div\");\n\n        ad.id = 'div-gpt-ad-1484122037505-9';\n        ad.style.height=\"250px\";\n        ad.style.width=\"300px;\";\n\n        inline.appendChild(ad);\n        inline.className = \"inline\";\n        var placeholder = document.getElementById(\"hidden-div-gpt-ad-1484122037505-9\");\n        placeholder.parentNode.replaceChild(inline, placeholder);\n        (function () {\n            googletag.cmd.push(function () {\n                googletag.display('div-gpt-ad-1484122037505-9');\n            });\n        })();\n    }\n<\/script>\n                    <\/div>\n<\/p><\/div>\n<\/p><\/div>\n<h2>\u00bfC\u00f3mo se diagnostica el s\u00edndrome de Chediak-Higashi?<\/h2>\n<p class=\"normal\">El diagn\u00f3stico del s\u00edndrome de Chediak-Higashi se realiza mediante:<\/p>\n<ul>\n<li>Examen de los gl\u00f3bulos blancos para detectar la presencia de gr\u00e1nulos grandes.<\/li>\n<li>Ligero <span class=\"term\" data-term-id=\"867\">microscop\u00eda<\/span> del cabello para encontrar <span class=\"term\" data-term-id=\"231\">pigmento<\/span> aglutinando.<\/li>\n<\/ul>\n<h2>\u00bfC\u00f3mo se trata el s\u00edndrome de Chediak-Higashi?<\/h2>\n<p class=\"normal\">El tratamiento para el s\u00edndrome de Chediak-Higashi puede incluir:<\/p>\n<ul>\n<li><span class=\"term\" data-term-id=\"1762\">Cable<\/span> sangre o m\u00e9dula \u00f3sea <span class=\"term\" data-term-id=\"1792\">trasplante<\/span>, que se considera el tratamiento de elecci\u00f3n;  tiene m\u00e1s \u00e9xito si se hace antes de que el ni\u00f1o alcance la fase acelerada de la enfermedad<\/li>\n<li><span class=\"term\" data-term-id=\"1619\">Quimioterapia<\/span> es necesario para poner la hemofagocitosis (la fase HLH) en <span class=\"term\" data-term-id=\"1260\">remisi\u00f3n<\/span> antes de que pueda tener lugar un trasplante<\/li>\n<li>Evitaci\u00f3n de la exposici\u00f3n a la luz solar y medidas de protecci\u00f3n solar.<\/li>\n<\/ul>\n<h2>Outlook \u00bfCu\u00e1l es la perspectiva del s\u00edndrome de Chediak-Higashi?<\/h2>\n<p class=\"normal\">Sin tratamiento, las perspectivas para el s\u00edndrome de Chediak-Higashi son malas.  Alrededor del 50 al 85% de los ni\u00f1os con este trastorno entrar\u00e1n en la fase acelerada de HLH.  Este proceso es fatal sin tratamiento y la mayor\u00eda de los pacientes morir\u00e1n a la edad de 10 a\u00f1os.<\/p>\n<\/section>\n","protected":false},"excerpt":{"rendered":"<p>\u00bfQu\u00e9 es Chediak-Higashi? s\u00edndrome? El s\u00edndrome de Chediak-Higashi es un trastorno infantil que interfiere con la funci\u00f3n del sistema inmunol\u00f3gico. Tambi\u00e9n se conoce como s\u00edndrome de Begnez-Cesar, leucocito anomal\u00eda albinismo o defecto en asesino natural linfocitos. Fue descrito por primera vez en 1943 por el Dr. Beguez-Cesar. \u00bfQui\u00e9n contrae el s\u00edndrome de Chediak-Higashi? El s\u00edndrome [&hellip;]<\/p>\n","protected":false},"author":1,"featured_media":4546,"parent":0,"template":"","glossary-cat":[],"class_list":["post-4544","glossary","type-glossary","status-publish","has-post-thumbnail","hentry"],"yoast_head":"<!-- This site is optimized with the Yoast SEO Premium plugin v26.2 (Yoast SEO v27.1.1) - https:\/\/yoast.com\/product\/yoast-seo-premium-wordpress\/ -->\n<title>S\u00edndrome de Chediak-Higashi - Dermatly.com - El sitio de tu piel<\/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:\/\/dermatly.com\/en\/chediak-higashi-syndrome\/\" \/>\n<meta property=\"og:locale\" content=\"en_US\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"S\u00edndrome de Chediak-Higashi\" \/>\n<meta property=\"og:description\" content=\"\u00bfQu\u00e9 es Chediak-Higashi? s\u00edndrome? El s\u00edndrome de Chediak-Higashi es un trastorno infantil que interfiere con la funci\u00f3n del sistema inmunol\u00f3gico. Tambi\u00e9n se conoce como s\u00edndrome de Begnez-Cesar, leucocito anomal\u00eda albinismo o defecto en asesino natural linfocitos. Fue descrito por primera vez en 1943 por el Dr. Beguez-Cesar. \u00bfQui\u00e9n contrae el s\u00edndrome de Chediak-Higashi? 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El s\u00edndrome de Chediak-Higashi es un trastorno infantil que interfiere con la funci\u00f3n del sistema inmunol\u00f3gico. Tambi\u00e9n se conoce como s\u00edndrome de Begnez-Cesar, leucocito anomal\u00eda albinismo o defecto en asesino natural linfocitos. Fue descrito por primera vez en 1943 por el Dr. Beguez-Cesar. \u00bfQui\u00e9n contrae el s\u00edndrome de Chediak-Higashi? 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