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HZ 10h45  11h00. Accueil  Introduction (P. Coppo) Travaux en cours: 11h00  11h30. PTTRitux2: analyse prliminaire (M. Buffet, Saint-Antoine) 11h30  12h00. Rupture de la tolrance du systme immunitaire vis--vis d ADAMTS13 dans le PTT acquis(L. Gilardin, Institut de Recherche des Cordeliers) 12h00  12h30. Dosage de l activit ADAMTS13 en Franceet bonnes pratiques : enqute nationale (A. Veyradier, Bclre/Bictre) Djeuner 13h30  14h00. L Association MAT ADAMTS13(S. Corset) Projets: 14h00  14h30. Rle des anticorps dans l activation de l endothlium au cours du PTT acquis: rsultats prliminaires. Quelles perspectives pour l utilisation d une cascade de filtration chez les malades les plus graves? (P. Poullin/G. Kaplanski, Marseille) 14h30  15h00. Peut-on appliquer la stratgie Pick a winner dans les MAT pour accroitre le nombre de nouveaux schmas thrapeutiques valuer ?(P. Coppo, Saint-Antoine) 15h00  15h30. Evaluation de l intrt du manganse dans les diarrhes STEC+ pour prvenir l volution vers un SHU/limiter la svrit du SHU: projet Mangashu (P. Galichon/A. Hertig, Tenon) 15h30  16h00. Evaluation de l intrt de l culizumab au cours des MAT de l allogreff: projet Soligreffe (R. Peffault Delatour, Saint-Louis) 16h00  16h30. Safety /tolerance of plasma inactivated by Amotosalen: study proposal (L. Corash, Cerus, USA) Pause 17h00  17h30. Projet MAT grossesse(Julie Peltier, Tenon/Yahsou Delmas, Bordeaux) 17h30  18h00. Etude pilote sur la vasculocomptence chez les patients recevant une allogreffe de cellules souches hmatopotiques (L. Gilardin, Institut de Recherche des Cordeliers)1" # "  #  m" # ;" # h "   , " $#$ ("( ,,  S F U  B   3  % .   D   4  R  ;    K E a     0     0PPp p /5 Runion du CNR MAT Vendredi 19 octobre 201200#   ,8  <$0  |Maison de l Amrique Latine; 217, Boulevard St Germain, Paris??# >H  0޽h ? ̙33h0 @(  p  0`@ JBilan d activit de l anne passe: Activit scientifique: Etudes publies : - Facteurs pronostiques dans le PTT acquis (Haematologica) - Traitements de sauvetage par Splnectomie et cyclophosphamide (Transfusion) - MAT chez sujet infect par le VIH (Revue de Mdecine Interne) - Etude des PTT chez la femme enceinte (Blood) - Pronostic des MAT (idiopathiques ou non) (Intensive Care Med) - Microangiopathies thrombotiques: physiopathologie et traitement (Revue du Praticien) Travaux en cours de rdaction/soumission: - Etude rtrospective des patients ayant reu du rituximab en prophylaxie Gnes de susceptibilit impliqus dans le PTT acquis (80 patients) (P. Loiseau/R. Tamouza/P. Coppo) - TLR2, TLR4, TLR9, MYD88 - TNFa, IFNg, IL6, IL10, TGFb, IL-4 et IL-4R - Etude des MAT avec activit ADAMTS13 dtectable (Q. Raimbourg, Tenon) - Facteurs dclenchants infectieux au cours des MAT (M. Levieil, Saint-Antoine) - Evaluation du plasma bleu de mthylne dans le PTT acquis (M. Hacquard/L. Chevreux/T. Schneider, Nancy) Etudes en cours: - Thromboses au cours des MAT (G. Sauvetre, Rouen) - Caractristiques cliniques et gnes de susceptibilit dans le PTT du sujet noir - Valeur pronostique des sous-classes d immunoglobuline (collaboration Baxter Inc.) - Consquences des transfusions plaquettaires au cours des PTT - Incidence des maladies systmiques/survenue/volution dans le PTT : comment les surveiller ? (M. Roriz, M. Landais, J. Desprez) Valeur pronostique de la troponine au diagnostic au cours des PTT acquis Protocoles en cours/ venir: - Mcanismes de la rupture de la tolrance du systme immunitaire v/v d ADAMTS13 - Intrt de l'culizumab au cours des MAT des allogreffes de CSH - Dmembrement des MAT au cours de la grossesse - Evaluation de l intrt du manganse dans les diarrhes STEC+ pour prvenir l volution vers un SHU/limiter la svrit du SHU: projet Mangashu -Etude pilote sur la vasculocomptence chez les patients recevant une allogreffe de cellules souches hmatopotiques (L. Gilardin, Institut de Recherche des Cordeliers) -Q-J-"-$cc"""""-" "C" "'""*"".""E"""+"""""""V"   l         H  N @ . K E R UH  0޽h ? ̙330 jb (     0ˮ6 bLe registre: > 1600 cas - Exhaustivit estime > 80% pour les MAT idiopathiques (PTT, SHU, autres) 2 portes d entre: patients signals Sandrine Malot; demandes d exploration d ADAMTS13: Laboratoire d Agns Veyradier Amliorer l exhaustivit du suivi; limiter les perdus de vue: Devenir des patients, histoire naturelle (autoimmunit), frquence des rechutes; Devenir des patientes en ge de procrer+++ Evolution des patients traits par rituximab Suggestion: mettre en copie des courriers de consultation Sandrine Malot+++ (Service d Hmatologie; Hpital Saint-Antoine - Paris)-B--A---#'3#'#"B""?"4"       wH  0޽h ? ̙33rPOd$ :AOh+'0x `h  Prsentation PowerPointpcoppog-sat-ohemapth6Microsoft PowerPoint@)X @|a@@nʂVGhg  ,V'&" WMFC <8?@ABCDEFGHIJKLMNOPQRSTUVWXYZ[\]^_`abcdefghijklmnopqrstuvwxyz{|}~      !"#$%&'()*+,-./0123456789:;<=>?@ABCDEFGHIJKLMNOPQRSTUVWXZ[\]^_`abcdefghijklmnopqrstuvwxyz|}~Root EntrydO)Pictures*Current UserSummaryInformation({PowerPoint Document(YBDocumentSummaryInformation8