{"id":83,"date":"2010-04-25T22:25:23","date_gmt":"2010-04-25T20:25:23","guid":{"rendered":"http:\/\/www.pjh.fr\/wordpress\/?page_id=83"},"modified":"2010-05-14T00:51:56","modified_gmt":"2010-05-13T22:51:56","slug":"explorations-a-realiser","status":"publish","type":"page","link":"http:\/\/www.pjh.fr\/wordpress\/?page_id=83","title":{"rendered":"Explorations \u00e0 r\u00e9aliser"},"content":{"rendered":"<h2>A. Explorations sanguines syst\u00e9matiques<\/h2>\n<ul>\n<li><strong>Electrophor\u00e8se  des protides, Immunofixation, dosage  pond\u00e9ral des Ig<\/strong><\/li>\n<\/ul>\n<p style=\"padding-left: 60px;\">En cas  de suspicion de <strong>cryoglobulin\u00e9mie<\/strong>, les  pr\u00e9l\u00e8vements doivent \u00eatre  transport\u00e9s au chaud (cela \u00e9vite une  sous-estimation du pic d&rsquo;IgM)<\/p>\n<blockquote><p><strong> L&rsquo;\u00e9lectrophor\u00e8se  doit \u00eatre id\u00e9alement surveill\u00e9e dans le  m\u00eame laboratoire.<\/strong><br \/>\nPas de  cha\u00eenes l\u00e9g\u00e8res libres en routine.<\/p><\/blockquote>\n<p style=\"padding-left: 60px;\">Les IgA et les IgG peuvent \u00eatre l\u00e9g\u00e8rement  diminu\u00e9es expliquant  des probl\u00e8mes r\u00e9currents d&rsquo;infections ORL ou  bronchiques. Leur niveau  revient rarement \u00e0 la normale m\u00eame \u00e0 distance  des traitements chez les  patients en r\u00e9mission compl\u00e8te.<\/p>\n<ul>\n<li><strong>NFS  + <\/strong><strong><span style=\"color: #ff0000;\">r\u00e9ticulocytes<\/span><\/strong><\/li>\n<li><strong><span style=\"color: #000000;\">+\/-<\/span> marqueurs d&rsquo;h\u00e9molyse : Haptoglobine,   Bilirubine T\/C, LDH <\/strong><\/li>\n<\/ul>\n<blockquote><p>VGM + r\u00e9ticulocytes afin de d\u00e9pister une h\u00e9molyse sous-jacente<\/p><\/blockquote>\n<ul>\n<li><strong>Fonction   r\u00e9nale : cr\u00e9atinin\u00e9mie <\/strong>(\u00e9tiologies des insuffisances r\u00e9nales :  cf  tableau 2)<\/li>\n<li><strong>Bilan h\u00e9patique : ASAT, ALAT, GGT,  PAlc, TP<\/strong><\/li>\n<li><strong>Beta2  microglobuline, Albumin\u00e9mie <\/strong>(int\u00e9r\u00eat  pronostic)<\/li>\n<\/ul>\n<h2>B. Examens selon signes d&rsquo;orientation<\/h2>\n<ul>\n<li><strong><span style=\"text-decoration: underline;\">Si an\u00e9mie<\/span> :<\/strong>\n<ul>\n<li>AHAI : recherche d&rsquo;<strong>agglutinines  froides<\/strong>, <strong>Coombs<\/strong><\/li>\n<li>Carence  en Fer : <strong>ferritin\u00e9mie <\/strong>+\/- bilan martial complet<br \/>\nLa carence en  fer est souvent n\u00e9glig\u00e9e. Elle est \u00e9galement  tr\u00e8s souvent r\u00e9sistante  au fer per os mais pas au <strong>FER par VOIE  INTRAVEINEUSE<\/strong><\/li>\n<li><strong>H\u00e9modilution<\/strong><\/li>\n<li><strong>Envahissement   m\u00e9dullaire<\/strong><\/li>\n<\/ul>\n<\/li>\n<\/ul>\n<ul>\n<li><strong><span style=\"text-decoration: underline;\">Si suspicion d&rsquo;hyperviscosit\u00e9 ou pic  monoclonal important<\/span> (protid\u00e9mie &gt; 100 g\/L ou pic &gt; 30 g\/L) :<\/strong>\n<ul>\n<li><strong>FOND D&rsquo;OEIL  +++: signes d&rsquo;hyperviscosit\u00e9 <\/strong>(h\u00e9morragies,  courant  granuleux&#8230;)<br \/>\nSyst\u00e9matique si Pic &gt; 30 g\/L<\/li>\n<li>Signes d&rsquo;hyperviscosit\u00e9 + Pic d&rsquo;IgM peu \u00e9lev\u00e9 =   Rechercher une <strong>cryoglobulin\u00e9mie<\/strong><\/li>\n<\/ul>\n<\/li>\n<\/ul>\n<ul>\n<li><strong><span style=\"text-decoration: underline;\">Neuropathie   p\u00e9riph\u00e9rique<\/span> : 20 \u00e0 25 % des patients !<\/strong>\n<ul>\n<li>Amylose ?   Cryoglobulin\u00e9mie ?<\/li>\n<li><strong>Neuropathie li\u00e9 au pic IgM ? <\/strong>\n<ul>\n<li><strong>IgM   anti MAG, Ac anti ganglioside M1 et Ac anti sulfatide<\/strong><br \/>\n(leur   absence n&rsquo;exclue pas le diagnostic : cible antig\u00e9nique non encore   identifi\u00e9e)<\/li>\n<li><strong>EMG (neuropathie d\u00e9my\u00e9l\u00e9nisante)<\/strong><\/li>\n<li><strong>Cryoglobulin\u00e9mie<\/strong><\/li>\n<\/ul>\n<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n<blockquote><p>La biopsie nerveuse doit \u00eatre   \u00e9vit\u00e9e en raison des fr\u00e9quentes complications<\/p><\/blockquote>\n<blockquote><p>Plus rare : une <strong>myopathie <\/strong>associ\u00e9e  \u00e0 une maladie de Waldenstr\u00f6m\u00a0 ? recherche  d&rsquo;anticorps <strong>IgM  antidecorin<\/strong><\/p><\/blockquote>\n<p><strong>Tableau : Etiologies des complications neurologiques, r\u00e9nales et de l&rsquo;an\u00e9mie dans la maladie de Waldenstr\u00f6m<\/strong><\/p>\n<table style=\"width: 90%;\" border=\"1\" cellspacing=\"0\" cellpadding=\"2\" align=\"center\">\n<thead>\n<tr style=\"background-color: #c2c2c2;\" align=\"center\">\n<td><strong>Neuropathie<\/strong><\/td>\n<td><strong>Insuffisance r\u00e9nale<\/strong><\/td>\n<td><strong>An\u00e9mie<\/strong><\/td>\n<\/tr>\n<\/thead>\n<tbody>\n<tr>\n<td>Cryoglobulin\u00e9mie<\/td>\n<td>Cryoglobulin\u00e9mie<\/td>\n<td>An\u00e9mie h\u00e9molytique auto-immune (AHAI)<\/p>\n<ul>\n<li>Agglutinines froides<\/li>\n<li>Auto-anticorps chauds<\/li>\n<\/ul>\n<\/td>\n<\/tr>\n<tr>\n<td>Amylose<\/td>\n<td>Amylose<\/td>\n<td>Carence en fer<\/td>\n<\/tr>\n<tr>\n<td>Neuropathie auto-immune<\/td>\n<td>D\u00e9p\u00f4ts d&rsquo;Ig<\/td>\n<td>H\u00e9modilution (pic \u00e9lev\u00e9)<\/td>\n<\/tr>\n<tr>\n<td><\/td>\n<td>Infiltration lymphomateuse r\u00e9nale<\/td>\n<td>Centrale : infiltration m\u00e9dullaire<\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n<p>&#8212;<\/p>\n<h2><strong>C. Explorations m\u00e9dullaire<\/strong><\/h2>\n<p><strong>&#8212;<br \/>\n<\/strong><\/p>\n<p><strong>Infiltration m\u00e9dullaire  quasi-constante<\/strong><br \/>\nDiagnostic  confirm\u00e9 par la mise en \u00e9vidence d&rsquo;une  infiltration m\u00e9dullaire par une  population cellulaire <strong>lymphoplasmocytaire<\/strong> compos\u00e9e de  petits lymphocytes pr\u00e9sentant des signes de diff\u00e9renciation   lymphoplasmocytaire.<\/p>\n<p>&#8212;<\/p>\n<p><a href=\"http:\/\/www.pjh.fr\/wordpress\/wp-content\/uploads\/2010\/04\/plasmacytoid-lymphoblast-100x3-website-arrow.jpg\" rel=\"lightbox[83]\"><img loading=\"lazy\" class=\"alignleft size-thumbnail wp-image-93\" style=\"margin-left: 5px; margin-right: 5px;\" title=\"plasmacytoid-lymphoblast-100x3-website-arrow\" src=\"http:\/\/www.pjh.fr\/wordpress\/wp-content\/uploads\/2010\/04\/plasmacytoid-lymphoblast-100x3-website-arrow-150x150.jpg\" alt=\"\" width=\"150\" height=\"150\" srcset=\"http:\/\/www.pjh.fr\/wordpress\/wp-content\/uploads\/2010\/04\/plasmacytoid-lymphoblast-100x3-website-arrow-150x150.jpg 150w, http:\/\/www.pjh.fr\/wordpress\/wp-content\/uploads\/2010\/04\/plasmacytoid-lymphoblast-100x3-website-arrow-36x36.jpg 36w, http:\/\/www.pjh.fr\/wordpress\/wp-content\/uploads\/2010\/04\/plasmacytoid-lymphoblast-100x3-website-arrow-110x110.jpg 110w\" sizes=\"(max-width: 150px) 100vw, 150px\" \/><\/a><a href=\"http:\/\/www.pjh.fr\/wordpress\/wp-content\/uploads\/2010\/04\/plasmacytoid-lymphocyte-100x-website-arrow.jpg\" rel=\"lightbox[83]\"><img loading=\"lazy\" class=\"alignright size-thumbnail wp-image-94\" title=\"plasmacytoid-lymphocyte-100x-website-arrow\" src=\"http:\/\/www.pjh.fr\/wordpress\/wp-content\/uploads\/2010\/04\/plasmacytoid-lymphocyte-100x-website-arrow-150x150.jpg\" alt=\"\" width=\"150\" height=\"150\" srcset=\"http:\/\/www.pjh.fr\/wordpress\/wp-content\/uploads\/2010\/04\/plasmacytoid-lymphocyte-100x-website-arrow-150x150.jpg 150w, http:\/\/www.pjh.fr\/wordpress\/wp-content\/uploads\/2010\/04\/plasmacytoid-lymphocyte-100x-website-arrow-36x36.jpg 36w, http:\/\/www.pjh.fr\/wordpress\/wp-content\/uploads\/2010\/04\/plasmacytoid-lymphocyte-100x-website-arrow-110x110.jpg 110w\" sizes=\"(max-width: 150px) 100vw, 150px\" \/><\/a><\/p>\n<p>Petits lymphocytes avec diff\u00e9renciation   plasmacyto\u00efde\/plasmocytaire(fl\u00e8ches)<\/p>\n<p>L&rsquo;infiltration m\u00e9dullaire   peut \u00eatre diffuse, interstitielle ou nodulaire et est habituellement   intertrab\u00e9culaire (une\u00a0 infiltration uniquement paratrab\u00e9culaire doit   faire \u00e9voquer le diagnostic diff\u00e9rentiel de lymphome folliculaire).<\/p>\n<p>L&rsquo;infiltration  m\u00e9dullaire doit \u00eatre \u00e9tay\u00e9e par des explorations  ph\u00e9notypiques  (cytom\u00e9trie de flux ou immunohistochimies).<\/p>\n<blockquote><p>Ph\u00e9notype classique : <strong>sIgM+ CD19+ CD20+ CD22+ CD79+<\/strong><\/p><\/blockquote>\n<blockquote><p>Classiquement   : CD10-, CD23-, CD138-, CD38 low ou (-).<br \/>\nJusqu&rsquo;\u00e0 20 % des cas   peuvent exprimer CD5, CD10 ou CD23 (diagnostic diff\u00e9rentiel : LLC,   lymphome du manteau).<\/p><\/blockquote>\n<p>Une augmentation du nombre de <strong> mastocytes<\/strong> associ\u00e9s \u00e0 l&rsquo;infiltrat lympho\u00efde est commun\u00e9ment retrouv\u00e9   dans la maladie de Waldenstr\u00f6m \u00e0 la diff\u00e9rence d&rsquo;autres lymphopathies B.<\/p>\n<h2>D.   Cytog\u00e9n\u00e9tique<\/h2>\n<p>D\u00e9l\u00e9tions 6q (jusqu&rsquo;\u00e0 50 % des cas) &#8211; Pas   d&rsquo;int\u00e9r\u00eat pronostic<\/p>\n<blockquote><p>La cytog\u00e9n\u00e9tique n&rsquo;a pas   d&rsquo;int\u00e9r\u00eat en routine actuellement sauf pour diff\u00e9rencier la maladie de   Waldenstr\u00f6m de rares cas de my\u00e9lomes \u00e0 IgM<\/p><\/blockquote>\n<h2>E. Imagerie<\/h2>\n<ul>\n<li> <strong>Scanner   thoraco-abdomino-pelvien<\/strong> (20 % de maladie extra-m\u00e9dullaire)\n<ul>\n<li>au   diagnostic<\/li>\n<li>au suivi si atteinte extra-m\u00e9dullaire initiale ou   suspect\u00e9e lors de l&rsquo;\u00e9volution<\/li>\n<\/ul>\n<\/li>\n<li>Pas de place \u00e0 l&rsquo;IRM<\/li>\n<li><strong>TEP <\/strong>: pas de place en routine, <strong>uniquement si suspicion de   transformation en lymphome de haut grade<br \/>\n<\/strong><\/li>\n<\/ul>\n<h2>F.  Fond  d&rsquo;oeil<\/h2>\n<p>Si suspicion de syndrome d&rsquo;hyperviscosit\u00e9<br \/>\n<strong>Syst\u00e9matique   si Pic &gt; 30 g\/L<\/strong><\/p>\n<p><strong>[table id =4 \/]<\/strong><br \/>\n<\/p>\n<div class=\"pdf24Plugin-cp\"> \t<form name=\"pdf24Form0\" method=\"post\" action=\"https:\/\/doc2pdf.pdf24.org\/wordpress.php\" target=\"pdf24PopWin\" onsubmit=\"var pdf24Win = window.open('about:blank', 'pdf24PopWin', 'resizable=yes,scrollbars=yes,width=600,height=250,left='+(screen.width\/2-300)+',top='+(screen.height\/3-125)+''); pdf24Win.focus(); if(typeof pdf24OnCreatePDF === 'function'){void(pdf24OnCreatePDF(this,pdf24Win));}\"> \t\t<input type=\"hidden\" name=\"blogCharset\" value=\"Cw1x07UAAA==\" \/><input type=\"hidden\" name=\"blogPosts\" value=\"MwQA\" \/><input type=\"hidden\" name=\"blogUrl\" value=\"yygpKbDS1y8vL9cryMrQSyvSL88vSikoSi0uBgA=\" \/><input type=\"hidden\" name=\"blogName\" value=\"C0gtKs7PS8xR8MovLQLR+WkKjgoeiam5iSX5OfnpmcUlAA==\" \/><input type=\"hidden\" 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Explorations sanguines syst\u00e9matiques Electrophor\u00e8se des protides, Immunofixation, dosage pond\u00e9ral des Ig En cas de suspicion de cryoglobulin\u00e9mie, les pr\u00e9l\u00e8vements doivent \u00eatre transport\u00e9s au chaud (cela \u00e9vite une sous-estimation du pic d&rsquo;IgM) L&rsquo;\u00e9lectrophor\u00e8se doit \u00eatre id\u00e9alement surveill\u00e9e dans le m\u00eame laboratoire. Pas de cha\u00eenes l\u00e9g\u00e8res libres en routine. Les IgA et les IgG peuvent \u00eatre [&hellip;]<\/p>\n","protected":false},"author":1,"featured_media":0,"parent":24,"menu_order":0,"comment_status":"open","ping_status":"open","template":"","meta":[],"_links":{"self":[{"href":"http:\/\/www.pjh.fr\/wordpress\/index.php?rest_route=\/wp\/v2\/pages\/83"}],"collection":[{"href":"http:\/\/www.pjh.fr\/wordpress\/index.php?rest_route=\/wp\/v2\/pages"}],"about":[{"href":"http:\/\/www.pjh.fr\/wordpress\/index.php?rest_route=\/wp\/v2\/types\/page"}],"author":[{"embeddable":true,"href":"http:\/\/www.pjh.fr\/wordpress\/index.php?rest_route=\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"http:\/\/www.pjh.fr\/wordpress\/index.php?rest_route=%2Fwp%2Fv2%2Fcomments&post=83"}],"version-history":[{"count":30,"href":"http:\/\/www.pjh.fr\/wordpress\/index.php?rest_route=\/wp\/v2\/pages\/83\/revisions"}],"predecessor-version":[{"id":358,"href":"http:\/\/www.pjh.fr\/wordpress\/index.php?rest_route=\/wp\/v2\/pages\/83\/revisions\/358"}],"up":[{"embeddable":true,"href":"http:\/\/www.pjh.fr\/wordpress\/index.php?rest_route=\/wp\/v2\/pages\/24"}],"wp:attachment":[{"href":"http:\/\/www.pjh.fr\/wordpress\/index.php?rest_route=%2Fwp%2Fv2%2Fmedia&parent=83"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}