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/Title (Relation entre la rponse hmatologique et l'volution des paramtres chographiques dans l'amylose AL avec atteinte cardiaque)
/Author (Martins Elie)
/ModDate (D:20161114111810+01'00')
/Subject (Introduction : Chez les patients prsentant une amylose AL, latteinte cardiaque reprsente un facteur pronostic majeur. Lefficacit du traitement est gnralement value par la rponse hmatologique, couple la rponse dorgane. Cependant, la relation entre la rponse hmatologique et lvaluation des paramtres chographiques post-traitement a t jusquici peu explore. Objectif : Analyser lvolution des paramtres chographiques sur le plan morphologique, fonctionnel et hmodynamique chez les patients prsentant une amylose AL avec atteinte cardiaque confirme, en fonction de leur rponse hmatologique post-chimiothrapie. Mthodes et rsultats : 63 patients ayant bnfici dune valuation chocardiographique initialement \(naf de tout traitement\) et aprs chimiothrapie ont t inclus dans notre tude. La rponse hmatologique tait dfinie selon 4 groupes : rponse complte \(normalisation du ratio des chanes lgres libres \(FLC\) et ngativation de limmunofixation srique et urinaire\), trs bonne rponse partielle \(diffrence entre le taux de la chane lgre monoclonale et celui de la chane lgre polyclonale \(dFLC\) <40mg/dL\), rponse partielle \(diminution de la dFLC >50%\) et absence de rponse \(diminution de la dFLC <50%\). Les patients avec une rponse complte ou une trs bonne rponse partielle taient classs rpondeurs tandis que les autres taient considrs non-rpondeurs. Parmi les 63 patients inclus \(ge moyen = 62,311,2 ans, 51% dhommes\), 38 \(60%\) taient rpondeurs et 25 \(40%\) considrs comme non-rpondeurs. A lexception dune proportion de tabagiques plus importante chez les non rpondeurs \(p=0,021\), il ny avait aucune diffrence significative entre les 2 groupes initialement sur les critres dmographiques, cliniques ou chographiques. Au suivi aprs chimiothrapie \(16,517,7 mois\), les rpondeurs diminuaient significativement leurs pressions artrielles pulmonaires systoliques en comparaison aux non rpondeurs \(p=0,04\). Chez les rpondeurs, lpaisseur de la paroi postrieure diminuait \(p=0,01\), les diamtres ventriculaires gauches tldiastoliques et tlsystoliques se majoraient \(p=0,012 et p=0,006 respectivement\) et la fraction djection du ventricule gauche \(FEVG\) tait plus basse \(p=0,005\). A loppos, les non-rpondeurs dgradaient significativement leur 2D-strain longitudinal global \(2D-GLS\) \(p=0,023\) et leur fonction systolique longitudinale du ventricule droit \(SVD\) \(p=0,008\). Par ailleurs, il existait une corrlation significative entre la FEVG, le 2D-GLS et certains paramtres de fonction diastolique mesurs initialement et le taux de NT-proBNP post-chimiothrapie. Lvolution du rapport E/E tait un facteur pronostic pour la survie globale. Conclusion : Lvolution des paramtres chographiques morphologiques, fonctionnels et hmodynamiques est associe la rponse hmatologique dans lamylose AL avec atteinte cardiaque. Lvaluation pr-chimiothrapie de la FEVG, du 2D-GLS et des paramtres de fonction diastolique pourrait reprsenter un lment pronostic intressant de rponse dorgane. )
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Martins Elie
Relation entre la réponse hématologique et l'évolution des paramètres échographiques dans l'amylose AL avec atteinte cardiaque
Introduction : Chez les patients présentant une amylose AL, l’atteinte cardiaque représente un facteur pronostic majeur. L’efficacité du traitement est généralement évaluée par la réponse hématologique, couplée à la réponse d’organe. Cependant, la relation entre la réponse hématologique et l’évaluation des paramètres échographiques post-traitement a été jusqu’ici peu explorée. Objectif : Analyser l’évolution des paramètres échographiques sur le plan morphologique, fonctionnel et hémodynamique chez les patients présentant une amylose AL avec atteinte cardiaque confirmée, en fonction de leur réponse hématologique post-chimiothérapie. Méthodes et résultats : 63 patients ayant bénéficié d’une évaluation échocardiographique initialement (naïf de tout traitement) et après chimiothérapie ont été inclus dans notre étude. La réponse hématologique était définie selon 4 groupes : réponse complète (normalisation du ratio des chaînes légères libres (FLC) et négativation de l’immunofixation sérique et urinaire), très bonne réponse partielle (différence entre le taux de la chaîne légère monoclonale et celui de la chaîne légère polyclonale (dFLC) <40mg/dL), réponse partielle (diminution de la dFLC >50%) et absence de réponse (diminution de la dFLC <50%). Les patients avec une réponse complète ou une très bonne réponse partielle étaient classés répondeurs tandis que les autres étaient considérés non-répondeurs. Parmi les 63 patients inclus (âge moyen = 62,3±11,2 ans, 51% d’hommes), 38 (60%) étaient répondeurs et 25 (40%) considérés comme non-répondeurs. A l’exception d’une proportion de tabagiques plus importante chez les non répondeurs (p=0,021), il n’y avait aucune différence significative entre les 2 groupes initialement sur les critères démographiques, cliniques ou échographiques. Au suivi après chimiothérapie (16,5±17,7 mois), les répondeurs diminuaient significativement leurs pressions artérielles pulmonaires systoliques en comparaison aux non répondeurs (p=0,04). Chez les répondeurs, l’épaisseur de la paroi postérieure diminuait (p=0,01), les diamètres ventriculaires gauches télédiastoliques et télésystoliques se majoraient (p=0,012 et p=0,006 respectivement) et la fraction d’éjection du ventricule gauche (FEVG) était plus basse (p=0,005). A l’opposé, les non-répondeurs dégradaient significativement leur 2D-strain longitudinal global (2D-GLS) (p=0,023) et leur fonction systolique longitudinale du ventricule droit (S’VD) (p=0,008). Par ailleurs, il existait une corrélation significative entre la FEVG, le 2D-GLS et certains paramètres de fonction diastolique mesurés initialement et le taux de NT-proBNP post-chimiothérapie. L’évolution du rapport E/E’ était un facteur pronostic pour la survie globale. Conclusion : L’évolution des paramètres échographiques morphologiques, fonctionnels et hémodynamiques est associée à la réponse hématologique dans l’amylose AL avec atteinte cardiaque. L’évaluation pré-chimiothérapie de la FEVG, du 2D-GLS et des paramètres de fonction diastolique pourrait représenter un élément pronostic intéressant de réponse d’organe.
2016-11-14T11:18:10+01:00
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