{"id":15642,"date":"2021-12-14T07:00:55","date_gmt":"2021-12-14T06:00:55","guid":{"rendered":"https:\/\/convergences.online\/hemato\/?p=15642"},"modified":"2024-12-11T14:25:48","modified_gmt":"2024-12-11T13:25:48","slug":"syndrome-vexas-et-manifestations-hematologiques","status":"publish","type":"post","link":"https:\/\/www.hematostat.net\/en\/syndrome-vexas-et-manifestations-hematologiques\/","title":{"rendered":"Syndrome VEXAS et manifestations h\u00e9matologiques"},"content":{"rendered":"<div class=\"fusion-fullwidth fullwidth-box fusion-builder-row-1 fusion-flex-container nonhundred-percent-fullwidth non-hundred-percent-height-scrolling\" style=\"--awb-border-radius-top-left:0px;--awb-border-radius-top-right:0px;--awb-border-radius-bottom-right:0px;--awb-border-radius-bottom-left:0px;--awb-flex-wrap:wrap;\" ><div class=\"fusion-builder-row fusion-row fusion-flex-align-items-flex-start fusion-flex-content-wrap\" style=\"max-width:1302px;margin-left: calc(-5% \/ 2 );margin-right: calc(-5% \/ 2 );\"><div class=\"fusion-layout-column fusion_builder_column fusion-builder-column-0 fusion_builder_column_1_1 1_1 fusion-flex-column\" style=\"--awb-bg-size:cover;--awb-width-large:100%;--awb-margin-top-large:0px;--awb-spacing-right-large:2.375%;--awb-margin-bottom-large:0px;--awb-spacing-left-large:2.375%;--awb-width-medium:100%;--awb-spacing-right-medium:2.375%;--awb-spacing-left-medium:2.375%;--awb-width-small:100%;--awb-spacing-right-small:2.375%;--awb-spacing-left-small:2.375%;\"><div class=\"fusion-column-wrapper fusion-flex-justify-content-flex-start fusion-content-layout-column\"><div class=\"fusion-text fusion-text-1\"><p>R\u00e9f. : HematoStat.net ; 2 (4) : R36<\/p>\n<p><em>Obiorah IE et al. Benign and malignant hematologic manifestations in patients with VEXAS syndrome due to somatic mutations in UBA1. <\/em><em>Blood Adv. 24 ao\u00fbt 2021;5(16):3203<\/em><em>\u2011<\/em><em>15.<\/em><em> <\/em><\/p>\n<p><strong>R\u00e9sum\u00e9 de l&#8217;article<\/strong><\/p>\n<p>Focus h\u00e9matologique et description d\u2019une cohorte de 16 patients atteints du syndrome VEXAS (vacuoles, enzyme E1, lien \u00e0 l\u2019X, auto-inflammation, somatique). Dix patients pr\u00e9sentent une h\u00e9mopathie au sens large (6 syndromes my\u00e9lodysplasiques, 2 MGUS, 2 lymphocytoses B monoclonales). Une an\u00e9mie macrocytaire \u00e9tait retrouv\u00e9e chez tous les patients. Enfin, 10 patients sur 16 ont pr\u00e9sent\u00e9 un \u00e9v\u00e9nement thrombotique (veineux ou art\u00e9riel).<\/p>\n<p><strong>Dans nos pratiques<\/strong><\/p>\n<p>Le syndrome VEXAS, r\u00e9cemment d\u00e9crit, r\u00e9sulte d\u2019une mutation somatique d\u2019UBA1 dans la cellule souche h\u00e9matopo\u00ef\u00e9tique. Touchant les hommes \u00e0 partir de 45 ou 50 ans, le tableau est domin\u00e9 par de la fi\u00e8vre, des atteintes rhumatologique et la pr\u00e9sence de vacuoles cytoplasmiques dans les pr\u00e9curseurs h\u00e9matopo\u00ef\u00e9tiques au niveau m\u00e9dullaire.<\/p>\n<p>Une proportion importante de patients semble \u00e9galement progresser vers une h\u00e9mopathie, ce qui justifie une surveillance h\u00e9matologique attentive.<\/p>\n<p><strong>Le regard du statisticien<\/strong><\/p>\n<p>Le niveau de statistique dans cette publication est tr\u00e8s basique, se limitant \u00e0 des graphiques simples et quelques tests statistiques usuels. Tellement basique que l\u2019on peut constater deux erreurs ou manquements :<\/p>\n<p>1 &#8211; les tests utilis\u00e9s pour les comparaisons SMD <em>vs <\/em>non SMD sont d\u2019apr\u00e8s la l\u00e9gende des <em>\u00ab non parametric Student t test<\/em> \u00bb, ce qui n\u2019existe pas, ce sont des tests U de Mann-Whitney (on peut se demander comment les <em>reviewers <\/em>n\u2019ont pas d\u00e9cel\u00e9 cette erreur ?) ;<\/p>\n<p>2 &#8211; il manque les \u00e9carts-types ou les quartiles sur la figure pr\u00e9sentant l\u2019\u00e9volution de l\u2019h\u00e9moglobine et des plaquettes au fil du temps, sans non plus pr\u00e9ciser si ce sont les m\u00e9dianes ou les moyennes qui sont reli\u00e9es.<\/p>\n<\/div><\/div><\/div><\/div><\/div>","protected":false},"excerpt":{"rendered":"","protected":false},"author":16,"featured_media":15643,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"_monsterinsights_skip_tracking":false,"_monsterinsights_sitenote_active":false,"_monsterinsights_sitenote_note":"","_monsterinsights_sitenote_category":0,"_uf_show_specific_survey":0,"_uf_disable_surveys":false,"footnotes":""},"categories":[36],"tags":[],"ppma_author":[456],"class_list":["post-15642","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-revue-de-presse","author-alexis-genthon"],"aioseo_notices":[],"authors":[{"term_id":456,"user_id":16,"is_guest":0,"slug":"alexis-genthon","display_name":"Alexis GENTHON","avatar_url":{"url":"https:\/\/www.hematostat.net\/wp-content\/uploads\/2023\/06\/Capture-decran-2021-05-07-a-14.25.02.png","url2x":"https:\/\/www.hematostat.net\/wp-content\/uploads\/2023\/06\/Capture-decran-2021-05-07-a-14.25.02.png"},"first_name":"","last_name":"","user_url":"","description":"H\u00e9matologue.\r\nCorrespondance : H\u00f4pital Saint-Antoine \r\nService h\u00e9matologie clinique\r\n184 rue du Faubourg Saint-Antoine 75012 Paris."}],"_links":{"self":[{"href":"https:\/\/www.hematostat.net\/en\/wp-json\/wp\/v2\/posts\/15642","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/www.hematostat.net\/en\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/www.hematostat.net\/en\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/www.hematostat.net\/en\/wp-json\/wp\/v2\/users\/16"}],"replies":[{"embeddable":true,"href":"https:\/\/www.hematostat.net\/en\/wp-json\/wp\/v2\/comments?post=15642"}],"version-history":[{"count":1,"href":"https:\/\/www.hematostat.net\/en\/wp-json\/wp\/v2\/posts\/15642\/revisions"}],"predecessor-version":[{"id":18205,"href":"https:\/\/www.hematostat.net\/en\/wp-json\/wp\/v2\/posts\/15642\/revisions\/18205"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/www.hematostat.net\/en\/wp-json\/"}],"wp:attachment":[{"href":"https:\/\/www.hematostat.net\/en\/wp-json\/wp\/v2\/media?parent=15642"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/www.hematostat.net\/en\/wp-json\/wp\/v2\/categories?post=15642"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/www.hematostat.net\/en\/wp-json\/wp\/v2\/tags?post=15642"},{"taxonomy":"author","embeddable":true,"href":"https:\/\/www.hematostat.net\/en\/wp-json\/wp\/v2\/ppma_author?post=15642"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}