{"id":1995,"date":"2015-10-01T01:00:00","date_gmt":"2015-10-01T00:00:00","guid":{"rendered":"https:\/\/angh.net\/abstracts\/cas-clinique-une-jaunisse-mal-placee-ou-savoir-realiser-le-bon-geste\/"},"modified":"2018-10-20T18:40:12","modified_gmt":"2018-10-20T16:40:12","slug":"cas-clinique-une-jaunisse-mal-placee-ou-savoir-realiser-le-bon-geste","status":"publish","type":"post","link":"https:\/\/angh.net\/abstracts\/cas-clinique-une-jaunisse-mal-placee-ou-savoir-realiser-le-bon-geste\/","title":{"rendered":"Cas clinique : Une jaunisse mal plac\u00e9e (ou savoir r\u00e9aliser le bon geste \u2026)."},"content":{"rendered":"

2015<\/strong><\/p>\n

Berthaux J, Zanditenas D, Condat B, Bonnet J, Ould-Ahmed B, Ngo Y, Blazquez M, Service d\u2019h\u00e9pato-gastro-ent\u00e9rologie de l\u2019h\u00f4pital Saint-Camille, Bry-Sur-Marne<\/em><\/p>\n

H\u00e9patologie <\/strong>– \u00a02015-04-28 –\u00a0CC –<\/p>\n

________________________________<\/p>\n

M B., 80 ans, consultait en f\u00e9vrier 2012 pour un ict\u00e8re et un l\u00e9ger prurit apparu 1 mois auparavant, sans autre sympt\u00f4me. Il n’avait aucun ant\u00e9c\u00e9dent m\u00e9dical, ne prenait pas de traitement et ne d\u00e9clarait aucune intoxication alcoolo-tabagique.L’examen clinique \u00e9tait normal. Les tests h\u00e9patiques \u00e9taient tr\u00e8s perturb\u00e9s avec notamment une cholestase majeure (GGT \u00e0 15N, PAL \u00e0 20N, Bilirubine totale \u00e0 234 μmol\/L et conjugu\u00e9e \u00e0 157 μmol\/L, ALAT \u00e0 11N, ASAT \u00e0 7N). Le reste du bilan biologique (ionogramme sanguin, facteur V, fonction r\u00e9nale, \u00e9lectrophor\u00e8se des protides, albumin\u00e9mie \u2026) s’av\u00e9rerait normal. Les s\u00e9rologies virales A, B, C et E \u00e9taient n\u00e9gatives ainsi que les anticorps anti tissu (FAN, AC anti mitochondrie, muscle lisse, DNA, LKM1, CL1 et SLA), la cupr\u00e9mie et c\u00e9rul\u00e9oplasmine n\u2019apportaient pas d\u2019argument en faveur d\u2019une maladie de Wilson. L\u2019\u00e9chographie h\u00e9patique, le scanner thoraco-abdomino-pelvien, l\u2019angio-IRM h\u00e9patique, la Bili-IRM et l\u2019\u00e9cho-endoscopie ne montraient qu’une h\u00e9patom\u00e9galie mod\u00e9r\u00e9e et homog\u00e8ne.
\nAu 2\u00e8me mois, en raison de l’absence de diagnostic et devant l’aggravation de l’ict\u00e8re (Bilirubine conjugu\u00e9e \u00e0 400 μmol\/L), une biopsie h\u00e9patique de bonne taille \u00e9tait r\u00e9alis\u00e9e et adress\u00e9e en centre expert. L\u2019histologie h\u00e9patique \u00e9tait normale en dehors d’une cholestase marqu\u00e9.
\nAu 3\u00e8me mois, apr\u00e8s r\u00e9p\u00e9tition infructueuse des examens, l’ict\u00e8re continuait \u00e0 s’aggraver (Bilirubine conjugu\u00e9e \u00e0 500 μmol\/L) et apparaissait une anorexie. Une seconde biopsie h\u00e9patique \u00e9tait r\u00e9alis\u00e9e et \u00e0 nouveau adress\u00e9e en centre expert. L\u2019histologie h\u00e9patique \u00e9tait \u00e0 nouveau normale en dehors de la cholestase et d\u2019un foyer minime d’h\u00e9matopo\u00ef\u00e8se intra sinuso\u00efdale.
\nAu 5\u00e8me mois, alors que l\u2019ict\u00e8re persistait, apparaissait, une an\u00e9mie macrocytaire ar\u00e9g\u00e9n\u00e9rative (H\u00e9moglobine \u00e0 9.5g\/dL, VGM \u00e0105fl). L’absence de carences vitaminiques et de syndrome inflammatoire ainsi que l’h\u00e9matopo\u00ef\u00e8se extra m\u00e9dullaire observ\u00e9e sur l\u2019histologie h\u00e9patique, faisaient poser l\u2019indication d\u2019une Biopsie Ost\u00e9o-M\u00e9dullaire \u00e0 la recherche d’une cause centrale. Il \u00e9tait mis en \u00e9vidence une my\u00e9lofibrose diffuse, avec envahissement par des cellules types ad\u00e9nocarcinome \u00e9voquant la m\u00e9tastase m\u00e9dullaire d’un cancer r\u00e9nal a cellules claires.
\nUne cause originale pour cet ict\u00e8re majeur \u00e9tait enfin \u00e9voqu\u00e9e : cholestase paran\u00e9oplasique accompagnant un ad\u00e9nocarcinome r\u00e9nal \u00e0 cellule claire, le syndrome de STAUFFER. Cependant 2 atypies incontournables demeuraient: le bilan morphologique ne montrait aucune tumeur r\u00e9nale et le syndrome de Stauffer correspond \u00e0 une cholestase non ict\u00e9rique.
\nPour r\u00e9pondre \u00e0 la premi\u00e8re atypie, une autre tumeur primitive a cellules claires, notamment thyro\u00efde ou pulmonaire \u00e9tait recherch\u00e9e en vain. Le Pet TDM au18 FDG n’apportait pas la r\u00e9ponse. Malgr\u00e9 une \u00e9chographie prostatique normale, le PSA \u00e9tant \u00e9lev\u00e9 (138 UI\/L), une cartographie prostatique par biopsie endo-rectale \u00e9tait alors r\u00e9alis\u00e9e. Elle confirmait l’origine prostatique de cet ad\u00e9nocarcinome m\u00e9tastatique \u00e0 cellules claires.
\nUne castration chirurgicale \u00e9tait alors r\u00e9alis\u00e9e par pulpectomie. Deux mois plus tard le PSA \u00e9tait normalis\u00e9. 4 semaines apr\u00e8s la pulpectomie les anomalies des tests h\u00e9patiques commen\u00e7aient \u00e0 d\u00e9cro\u00eetre et se normalisaient en 12 semaines.
\nAfin d\u2019\u00e9tayer le r\u00f4le du syndrome paran\u00e9oplasique dans cet ict\u00e8re, un dosage r\u00e9trospectif de l’IL6 sur s\u00e9rum pr\u00e9-traitement (M2 de l\u2019ict\u00e8re) et post-traitement (M3 post traitement) \u00e9tait r\u00e9alis\u00e9. Le taux initial \u00e9tait de 8,9 pg\/ml (pour des valeurs usuelle < 5 ng\/ml), et le taux post traitement inf\u00e9rieur aux seuil de d\u00e9tection (< 0,2 ng\/ml).
\nAu final, cet ict\u00e8re inqui\u00e9tant et prolong\u00e9 a \u00e9t\u00e9 gu\u00e9ri par …… une pulpectomie testiculaire. <\/p>\n

DISCUSSION
\nLe syndrome de Stauffer est un syndrome paran\u00e9oplasique entra\u00eenant une cholestase intrah\u00e9patique habituellement anict\u00e9rique. Il est d\u00e9crit dans pr\u00e8s de 15% des ad\u00e9nocarcinomes r\u00e9naux \u00e0 cellules claires. Dans notre cas, Il s'agit d'un syndrome de Stauffer inhabituel pour 2 raisons : (1) Il n'est pas associ\u00e9 \u00e0 un cancer du rein mais \u00e0 une forme rare de cancer de la prostate dont l\u2019histologie \u00e0 cellules claires s'apparente \u00e0 celle des cancers du rein. Elle ne repr\u00e9sente moins de 1 % des cancers de la prostate ; (2) La forme ict\u00e9rique a d\u00e9j\u00e0 \u00e9t\u00e9 d\u00e9crite dans des cas de cancer du rein g\u00e9ant ; l'hypers\u00e9cr\u00e9tion paran\u00e9oplasique d'IL6 est probablement proportionnelle \u00e0 la masse tumorale. Pour notre patient, la masse tumorale \u00e9tait principalement repr\u00e9sent\u00e9e par l'envahissement m\u00e9dullaire ce qui repr\u00e9sente au final un volume tr\u00e8s important. L'effondrement des taux plasmatiques de l'IL6 synchrone avec le contr\u00f4le de la maladie et la disparition de l'ict\u00e8re permettent d'affirmer ce diagnostic et renforce l'hypoth\u00e8se d'un r\u00f4le majeur de cette cytokine dans la physiopathologie du syndrome de Stauffer en plus de son r\u00f4le inflammatoire, an\u00e9miant et thrombop\u00e9niant.
\nMais l\u2019enseignement principal de cette observation est que la m\u00e9decine est parfois surprenante !<\/p>\n","protected":false},"excerpt":{"rendered":"

2015 Berthaux J, Zanditenas D, Condat B, Bonnet J, Ould-Ahmed B, Ngo Y, Blazquez M, Service d\u2019h\u00e9pato-gastro-ent\u00e9rologie de l\u2019h\u00f4pital Saint-Camille, Bry-Sur-Marne H\u00e9patologie – \u00a02015-04-28 –\u00a0CC – ________________________________ M B., 80 ans, consultait en f\u00e9vrier 2012 pour un ict\u00e8re et un l\u00e9ger prurit apparu 1 mois auparavant, sans autre sympt\u00f4me. Il n’avait aucun ant\u00e9c\u00e9dent m\u00e9dical, ne […]<\/p>\n","protected":false},"author":2,"featured_media":0,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"_exactmetrics_skip_tracking":false,"_exactmetrics_sitenote_active":false,"_exactmetrics_sitenote_note":"","_exactmetrics_sitenote_category":0,"footnotes":""},"categories":[3],"tags":[26],"class_list":["post-1995","post","type-post","status-publish","format-standard","hentry","category-hepatologie","tag-26"],"yoast_head":"\nCas clinique : Une jaunisse mal plac\u00e9e (ou savoir r\u00e9aliser le bon geste \u2026). - Abstracts des congr\u00e8s de l'ANGH<\/title>\n<meta name=\"description\" content=\"ANGH R\u00e9sum\u00e9s congr\u00e8s H\u00e9patologie Gastroent\u00e9romogie Hepatology Gastroenterology Etudes cliniques Clinical study\" \/>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/angh.net\/abstracts\/cas-clinique-une-jaunisse-mal-placee-ou-savoir-realiser-le-bon-geste\/\" \/>\n<meta property=\"og:locale\" content=\"fr_FR\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"Cas clinique : Une jaunisse mal plac\u00e9e (ou savoir r\u00e9aliser le bon geste \u2026). - 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