Author: "Gérard Couillault" - Searchworks@Jio Institute Digital Library Search Results

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27 results on '"Gérard Couillault"'

1. An appraisal of the frequency and severity of noninfectious manifestations in primary immunodeficiencies: A study of a national retrospective cohort of 1375 patients over 10 years

Author: Mickaël Alligon, Nizar Mahlaoui, Virginie Courteille, Laurence Costes, Veronica Afonso, Philippe Randrianomenjanahary, Nathalie de Vergnes, Anja Ranohavimparany, Duy Vo, Inès Hafsa, Perrine Bach, Vincent Benoit, Nicolas Garcelon, Alain Fischer, Wadih Abou-Chahla, Daniel Adoue, Nathalie Aladjidi, Corinne Armari-Alla, Vincent Barlogis, Sophie Bayart, Yves Bertrand, Stéphane Blanche, Damien Bodet, Bernard Bonnotte, Raphaël Borie, Patrick Boutard, David Boutboul, Claire Briandet, Jean-Paul Brion, Jacques Brouard, Liana Carausu, Martin Castelle, Pascal Cathebras, Emilie Catherinot, Nathalie Cheikh, Morgane Cheminant, Sarah Cohen-Beaussant, Thibault Comont, Louis-Jean Couderc, Pierre Cougoul, Gérard Couillault, Lionel Crevon, Elisa Demonchy, Anne Deville, Catherine Devoldere, Eric Dore, Fabienne Dulieu, Isabelle Durieu, Natacha Entz-Werle, Claire Fieschi, Fanny Fouyssac, Pierre Frange, Vincent Gajdos, Lionel Galicier, Virginie Gandemer, Martine Gardembas, Catherine Gaud, Bernard Grosbois, Aurélien Guffroy, Corinne Guitton, Gaëlle Guillerm, Eric Hachulla, Mohamed Hamidou, Sophie Haro, Yves Hatchuel, Olivier Hermine, Cyrille Hoarau, Arnaud Hot, Sébastien Humbert, Arnaud Jaccard, Jean-Philippe Jais, Sarah Jannier, Serge Jacquot, Roland Jaussaud, Pierre-Yves Jeandel, Eric Jeziorski, Kamila Kebaili, Anne-Sophie Korganow, Olivier Lambotte, Fanny Lanternier, Claire Larroche, David Launay, Emmanuelle Le Moigne, Alain Le Quellec, Vincent Le Moing, Yvon Lebranchu, Marc Lecuit, Guillaume Lefèvre, Jean-Daniel Lelièvre, Richard Lemal, Valérie Li-Thiao-Te, Olivier Lortholary, Luminita Luca, Coralie Mallebranche, Marion Malphettes, Aude Marie-Cardine, Nicolas Martin-Silva, Agathe Masseau, Françoise Mazingue, Etienne Merlin, Gérard Michel, Frédéric Millot, Charline Miot, Béatrice Monlibert, Fabrice Monpoux, Despina Moshous, Luc Mouthon, Martine Münzer, Robert Navarro, Bénédicte Neven, Dalila Nouar, Raphaële Nove-Josserand, Eric Oksenhendler, Marie Ouachée-Chardin, Anne Pagnier, Marlène Pasquet, Isabelle Pellier, Yves Perel, Antoinette Perlat, Christophe Piguet, Dominique Plantaz, Sophie Rivière, Pascal Roblot, Pierre-Simon Rohrlich, Bruno Royer, Valéry Salle, Françoise Sarrot-Reynauld, Amélie Servettaz, Jean-Louis Stephan, Nicolas Schleinitz, Harry Sokol, Felipe Suarez, Laure Swiader, Sophie Taque, Caroline Thomas, Olivier Tournilhac, Caroline Thumerelle, Jean-Pierre Vannier, and Jean-François Viallard
Subjects: Inflammation, Neoplasms, Immunology, Hypersensitivity, Immunologic Deficiency Syndromes, Immunology and Allergy, Humans, Autoimmunity, Retrospective Studies
Abstract: Noninfectious manifestations-allergy, autoimmunity/inflammation, lymphoproliferation, and malignancies-are known to exist in many primary immunodeficiency diseases (PID) and to participate in prognosis.To obtain a global view on their occurrence, we retrieved data from a retrospective cohort of 1375 patients included in the French National Reference Center for Primary Immune Deficiencies (CEREDIH) for whom we had a 10-year follow-up since inclusion in the registry.These patients were followed for 10 years (2009-2018) by specialized centers in university hospitals. This study showed that 20.1% of patients without prior curative therapy (n = 1163) developed at least 1 manifestation (event) encompassing 277 events.Autoimmune/inflammatory events (n = 138) and malignancies (n = 85) affected all age classes and virtually all PID diagnostic groups. They were associated with a risk of death that occurred in 195 patients (14.2%) and were found to be causal in 43% of cases. Malignancies (odds ratio, 5.62; 95% confidence interval, 3.66-8.62) and autoimmunity (odds ratio, 1.9; 95% confidence interval, 1.27-2.84) were clearly identified as risk factors for lethality. Patients who underwent curative therapy (mostly allogeneic hematopoietic stem cell transplantation, with a few cases of gene therapy or thymus transplantation) before the 10-year study period (n = 212) had comparatively reduced but still detectable clinical manifestations (n = 16) leading to death in 9.4% of them.This study points to the frequency and severity of noninfectious manifestations in various PID groups across all age groups. These results warrant further prospective analysis to better assess their consequences and to adapt therapy, notably indication of curative therapy.
Published: 2021

2. Genetic diagnosis of primary immunodeficiencies: A survey of the French national registry

Author: Nizar Mahlaoui, Capucine Picard, Perrine Bach, Laurence Costes, Virginie Courteille, Anja Ranohavimparany, Alexandre Alcaïs, Jean-Philippe Jais, Alain Fischer, Christine Bellanné-Chantelot, Jacinta Bustamante, Sylvie Chollet-Martin, Christian Drouet, Véronique Fremeaux-Bacchi, Caroline Kannengiesser, Virginie Girardin, Nathalie Lambert, Valérie Proulle, Jérémie Rosain, Marie José Stasia, Dominique Stoppa Lyonnet, Ioannis Theodorou, Wadih Abou-Chahla, Daniel Adoue, Nathalie Aladjidi, Zahir Amoura, Corinne Armari-Alla, Brigitte Bader-Meunier, Vincent Barlogis, Sophie Bayart, Yves Bertrand, Stéphane Blanche, Damien Bodet, Bernard Bonnotte, Raphaël Borie, Patrick Boutard, Claire Briandet, Jean-Paul Brion, Jacques Brouard, Liana Carausu, Emilie Catherinot, Nathalie Cheikh, Sarah Cohen-Beaussant, Louis-Jean Couderc, Pierre Cougoul, Gérard Couillault, Geneviève de Saint Basile, Catherine Devoldere, Anne Deville, Eric Dore, Fabienne Dulieu, Isabelle Durieu, Natacha Entz Werle, Claire Fieschi, Fanny Fouyssac, Pierre Frange, Vincent Gajdos, Lionel Galicier, Virginie Gandemer, Martine Gardembas, Catherine Gaud, Bernard Grosbois, Aurélien Guffroy, Corinne Guitton, Gaelle Guillerm, Eric Hachulla, Mohamed Hamidou, Sophie Haro, Yves Hatchuel, Olivier Hermine, Cyrille Hoarau, Bruno Hoen, Arnaud Hot, Sébastien Humbert, Arnaud Jaccard, Serge Jacquot, Rolland Jaussaud, Pierre-Yves Jeandel, Eric Jeziorski, Kamila Kebaili, Anne-Sophie Korganow, Olivier Lambotte, Fanny Lanternier, Claire Larroche, Alain Le Quellec, Emmanuelle Le Moigne, Vincent Le Moing, David Launay, Yvon Lebranchu, Marc Lecuit, Guillaume Lefevre, Richard Lemal, Valérie Li-Thiao-Te, Olivier Lortholary, Marion Malphettes, Aude Marie-Cardine, Nicolas Martin Silva, Agathe Masseau, Christian Massot, Françoise Mazingue, Etienne Merlin, Gérard Michel, Frédéric Millot, Béatrice Monlibert, Fabrice Monpoux, Despina Moshous, Luc Mouthon, Martine Munzer, Bénédicte Neven, Raphaëlle Nove-Josserand, Dalila Nouar, Eric Oksenhendler, Marie Ouachée-Chardin, Anne Pagnier, Marlène Pasquet, Isabelle Pellier, Yves Perel, Antoinette Perlat, Christophe Piguet, Dominique Plantaz, Pierre Quartier, Frédéric Rieux-Laucat, Pascal Roblot, Pierre-Simon Rohrlich, Bruno Royer, Valéry Salle, Françoise Sarrot-Reynauld, Amélie Servettaz, Jean-Louis Stephan, Nicolas Schleinitz, Felipe Suarez, Laure Swiader, Sophie Taque, Caroline Thomas, Olivier Tournilhac, Caroline Thumerelle, Jean-Pierre Vannier, Jean-François Viallard, IFR Necker-Enfants Malades (IRNEM), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Imagine - Institut des maladies génétiques (IMAGINE - U1163), Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre de Référence Déficits Immunitaires Héréditaires (CEREDIH), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Nutrition et Neurobiologie intégrée (NutriNeuro), Université Bordeaux Segalen - Bordeaux 2-Institut National de la Recherche Agronomique (INRA)-Université Sciences et Technologies - Bordeaux 1 (UB)-Institut Polytechnique de Bordeaux-Ecole nationale supérieure de chimie, biologie et physique, Service de Biostatistique et Informatique Médicale, Université Paris Descartes - Paris 5 (UPD5), Service d'immuno-hématologie pédiatrique [CHU Necker], Collège de France - Chaire Médecine expérimentale (A. Fischer), Collège de France (CdF (institution)), CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Laboratoire d'Immunologie 'Autoimmunité et Hypersensibilités' [AP-HP Hôpital Bichat, Paris], AP-HP - Hôpital Bichat - Claude Bernard [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Groupe de Recherche et d'Etude du Processus Inflammatoire (GREPI), VetAgro Sup - Institut national d'enseignement supérieur et de recherche en alimentation, santé animale, sciences agronomiques et de l'environnement (VAS)-Centre National de la Recherche Scientifique (CNRS)-Université Grenoble Alpes [2016-2019] (UGA [2016-2019]), Service d'immunologie [HEGP, Paris], Hôpital Européen Georges Pompidou [APHP] (HEGP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO), Centre de recherche sur l'Inflammation (CRI (UMR_S_1149 / ERL_8252 / U1149)), Université Paris Diderot - Paris 7 (UPD7)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), the Study Center of Primary Immunodeficiencies, CHU Necker - Enfants Malades [AP-HP], Hémostase et biologie vasculaire, Université Paris-Sud - Paris 11 (UP11)-IFR93-Institut National de la Santé et de la Recherche Médicale (INSERM), Institut de biologie structurale (IBS - UMR 5075 ), Centre National de la Recherche Scientifique (CNRS)-Université Grenoble Alpes [2016-2019] (UGA [2016-2019])-Institut de Recherche Interdisciplinaire de Grenoble (IRIG), Direction de Recherche Fondamentale (CEA) (DRF (CEA)), Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Direction de Recherche Fondamentale (CEA) (DRF (CEA)), Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Commissariat à l'énergie atomique et aux énergies alternatives (CEA), Centre d'Immunologie et de Maladies Infectieuses (CIMI), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Institut Universitaire du Cancer de Toulouse - Oncopole (IUCT Oncopole - UMR 1037), Université Toulouse III - Paul Sabatier (UT3), Université de Toulouse (UT)-Université de Toulouse (UT)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Institut National de la Santé et de la Recherche Médicale (INSERM), Hôpital Pellegrin, CHU Bordeaux [Bordeaux]-Groupe hospitalier Pellegrin, Hôpital de la Tronche, Service d'hématologie pédiatrique, Université de la Méditerranée - Aix-Marseille 2-Assistance Publique - Hôpitaux de Marseille (APHM)- Hôpital de la Timone [CHU - APHM] (TIMONE), Hématogoie pédiatrique, hôpital Sud, SIGNAL-IMAGE-COMMUNICATION (SIC), Université de Poitiers-Centre National de la Recherche Scientifique (CNRS), Interactions hôte-greffon-tumeur, ingénierie cellulaire et génique - UFC (UMR INSERM 1098) (RIGHT), Institut National de la Santé et de la Recherche Médicale (INSERM)-Etablissement français du sang [Bourgogne-Franche-Comté] (EFS BFC)-Université de Franche-Comté (UFC), Université Bourgogne Franche-Comté [COMUE] (UBFC)-Université Bourgogne Franche-Comté [COMUE] (UBFC), Service de Pneumologie et Centre de Compétence des Maladies Pulmonaires Rares [AP-HP Hôpital Bichat, Paris], Hôpital Côte de Nacre [CHU Caen], CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN)-Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN), Service de Pédiatrie Enfants - Hématologie Oncologie [CHU de Dijon], Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Service des Maladies Infectieuses, CHU Grenoble, Service de Pédiatrie Médicale [Caen], Université de Caen Normandie (UNICAEN), Normandie Université (NU)-Normandie Université (NU)-CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN)-Tumorothèque de Caen Basse-Normandie (TCBN), Hôpital Foch [Suresnes], Service de pédiatrie (CHU de Dijon), Developpement Normal et Pathologique du Système Immunitaire, Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Centre Hospitalier Universitaire de Nice (CHU Nice), Centre d'Investigation Clinique [CHU Clermont-Ferrand] (CIC 1405), Institut National de la Santé et de la Recherche Médicale (INSERM)-Direction de la recherche clinique et de l’innovation [CHU Clermont-Ferrand] (DRCI), CHU Clermont-Ferrand-CHU Clermont-Ferrand, Centre Hospitalier Lyon Sud [CHU - HCL] (CHLS), Hospices Civils de Lyon (HCL), Immunologie clinique [CHU St-Louis], Groupe Hospitalier Saint Louis - Lariboisière - Fernand Widal [Paris], Service d'Hématologie et d'Oncologie Pédiatrique [CHRU Nancy], Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Infections à Vih, Réservoirs, Pharmacologie des Antirétroviraux et Prévention de la Transmission Mère Enfant, Centre de recherche en épidémiologie et santé des populations (CESP), Université de Versailles Saint-Quentin-en-Yvelines (UVSQ)-Université Paris-Sud - Paris 11 (UP11)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Paul Brousse-Institut National de la Santé et de la Recherche Médicale (INSERM), Institut de Génétique et Développement de Rennes (IGDR), Université de Rennes (UR)-Centre National de la Recherche Scientifique (CNRS)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Service de Médecine interne et immunologie clinique [Rennes] = internal medicine and clinical immunology [Rennes], CHU Pontchaillou [Rennes], Service d'Immunologie Clinique et de Médecine Interne, Centre National de Référence des Maladies Auto-immunes Rares, Hôpitaux Universitaires de Strasbourg, Service d'hématologie, immunologie biologiques et cytogénétique, Université Paris-Sud - Paris 11 (UP11)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Bicêtre, Service Hématologie, Hôpital Morvan [Brest]-Centre Hospitalier Régional Universitaire de Brest (CHRU Brest), Hôpital Claude Huriez [Lille], CHU Lille, Service de médecine interne [Nantes], Université de Nantes (UN)-Hôtel-Dieu-Centre hospitalier universitaire de Nantes (CHU Nantes), 'Personal Protection Against Vectors' working group (PPAV), PPAV working group, cellules dendritiques et greffes (JE 2448), Université de Tours (UT), Laboratoire Chrono-environnement (UMR 6249) (LCE), Centre National de la Recherche Scientifique (CNRS)-Université de Franche-Comté (UFC), Service de Médecine Interne, Hôpital Edouard Herriot [CHU - HCL], Hospices Civils de Lyon (HCL)-Hospices Civils de Lyon (HCL), Contrôle de la Réponse Immune B et des Lymphoproliférations (CRIBL), Université de Limoges (UNILIM)-Génomique, Environnement, Immunité, Santé, Thérapeutique (GEIST FR CNRS 3503)-Centre National de la Recherche Scientifique (CNRS), Physiopathologie et biothérapies des maladies inflammatoires et autoimmunes, Université de Rouen Normandie (UNIROUEN), Normandie Université (NU)-Normandie Université (NU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Médecine interne, maladies infectieuses, immunologie clinique, Hôpital Robert Debré-Centre Hospitalier Universitaire de Reims (CHU Reims), Institut de Génétique Moléculaire de Montpellier (IGMM), Université de Montpellier (UM)-Centre National de la Recherche Scientifique (CNRS), Institut d’Hémato-Oncologie Pédiatrique, INSERM U1012, AP-HP Hôpital Bicêtre (Le Kremlin-Bicêtre), Centre d'infectiologie Necker-Pasteur [CHU Necker], Institut Pasteur [Paris] (IP)-CHU Necker - Enfants Malades [AP-HP], Internal Medicine, Paris, Cellules Souches, Plasticité Cellulaire, Médecine Régénératrice et Immunothérapies (IRMB), Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Montpellier (UM), Département de Médecine Interne et Pneumologie [Brest] (DMIP - Brest), Centre Hospitalier Régional Universitaire de Brest (CHRU Brest), Département Maladies Infectieuses et Tropicales, Hôpital Universitaire, Montpellier, France, Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Service de médecine interne [Lille], Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Centre collaborateur de l'OMS Listeria / WHO Collaborating Centre Listeria (CC-OMS / WHO-CC), Institut Pasteur [Paris] (IP)-Organisation Mondiale de la Santé / World Health Organization Office (OMS / WHO), Laboratoire de Génie Electrique de Grenoble (G2ELab), Institut polytechnique de Grenoble - Grenoble Institute of Technology (Grenoble INP )-Centre National de la Recherche Scientifique (CNRS)-Université Grenoble Alpes [2016-2019] (UGA [2016-2019]), CHU Clermont-Ferrand, Service d'Immunopathologie [Hôpital Saint-Louis, Paris], Université Paris Diderot - Paris 7 (UPD7)-Hopital Saint-Louis [AP-HP] (AP-HP), Hôpital Charles Nicolle [Rouen], CHU Rouen, Normandie Université (NU)-Normandie Université (NU), Dept of Physiology, McGill University = Université McGill [Montréal, Canada], Franche-Comté Électronique Mécanique, Thermique et Optique - Sciences et Technologies (UMR 6174) (FEMTO-ST), Université de Technologie de Belfort-Montbeliard (UTBM)-Ecole Nationale Supérieure de Mécanique et des Microtechniques (ENSMM)-Centre National de la Recherche Scientifique (CNRS)-Université de Franche-Comté (UFC), Centre hospitalier universitaire de Poitiers (CHU Poitiers), Service de médecine interne et centre de référence des maladies rares [CHU Cochin], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Cochin [AP-HP], American Memorial Hospital, American Memory Hospital, Department of Clinical Immunology, Service d'hématologie et immunologie pédiatrique, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Robert Debré-Université Paris Diderot - Paris 7 (UPD7), Service Immuno Hémato-Onco Pédiatrique, Sercice Hématologie, immunologie et oncologie pédiatrique [CHU Toulouse], Pôle Enfants [CHU Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Service de Pédiatrie médicale - Spécialités médicales [CHU Limoges], CHU Limoges, Service Hématologie Infantile, CHU Amiens-Picardie, Clinique de médecine interne, Université Joseph Fourier - Grenoble 1 (UJF)-CHU Grenoble, Département de Médecine Interne, Immunologie clinique et maladies infectieuses [CHU Reims], Université de Reims Champagne-Ardenne (URCA)-Centre Hospitalier Universitaire de Reims (CHU Reims), Centre Hospitalier Universitaire de Saint-Etienne [CHU Saint-Etienne] (CHU ST-E), Centre d'Immunologie de Marseille - Luminy (CIML), Aix Marseille Université (AMU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Service de médecine de l'enfant et de l'adolescent [CHU Rennes], Clinical Haematology, CHU Hôtel-Dieu, Hôpital Haut-Lévêque, Université Sciences et Technologies - Bordeaux 1 (UB)-CHU Bordeaux [Bordeaux], French Ministry of Health, LFB, GlaxoSmithKlineGlaxoSmithKline, CSL BehringCSL Behring, ShireShire, Octapharma, Binding Site, Université Bordeaux Segalen - Bordeaux 2-Institut National de la Recherche Agronomique (INRA)-Université Sciences et Technologies - Bordeaux 1-Institut Polytechnique de Bordeaux-Ecole nationale supérieure de chimie, biologie et physique, Chaire Médecine expérimentale (A. Fischer), Service de Génétique Cytogénétique et Embryologie [CHU Pitié-Salpêtrière], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), GREPI, Techniques de l'Ingénierie Médicale et de la Complexité - Informatique, Mathématiques et Applications, Grenoble - UMR 5525 (TIMC-IMAG), VetAgro Sup - Institut national d'enseignement supérieur et de recherche en alimentation, santé animale, sciences agronomiques et de l'environnement (VAS)-Institut polytechnique de Grenoble - Grenoble Institute of Technology (Grenoble INP )-Centre National de la Recherche Scientifique (CNRS)-Université Joseph Fourier - Grenoble 1 (UJF)-VetAgro Sup - Institut national d'enseignement supérieur et de recherche en alimentation, santé animale, sciences agronomiques et de l'environnement (VAS)-Institut polytechnique de Grenoble - Grenoble Institute of Technology (Grenoble INP )-Centre National de la Recherche Scientifique (CNRS)-Université Joseph Fourier - Grenoble 1 (UJF), Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université Paris Diderot - Paris 7 (UPD7), Université Grenoble Alpes [2016-2019] (UGA [2016-2019])-Institut de Recherche Interdisciplinaire de Grenoble (IRIG), Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Centre National de la Recherche Scientifique (CNRS), Centre National de la Recherche Scientifique (CNRS)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Pierre et Marie Curie - Paris 6 (UPMC), CHU Toulouse [Toulouse]-Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de médecine interne [CHU Pitié-Salpétrière], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Franche-Comté (UFC), Université Bourgogne Franche-Comté [COMUE] (UBFC)-Université Bourgogne Franche-Comté [COMUE] (UBFC)-Etablissement français du sang [Bourgogne-Franche-Comté] (EFS [Bourgogne-Franche-Comté]), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris-Sud - Paris 11 (UP11)-Hôpital Paul Brousse-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Versailles Saint-Quentin-en-Yvelines (UVSQ), Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Centre National de la Recherche Scientifique (CNRS)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Laboratoire Chrono-environnement - CNRS - UBFC (UMR 6249) (LCE), Institut Pasteur [Paris]-CHU Necker - Enfants Malades [AP-HP], Université de Montpellier (UM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Institut Pasteur [Paris]-Organisation Mondiale de la Santé / World Health Organization Office (OMS / WHO), Hopital Saint-Louis [AP-HP] (AP-HP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Diderot - Paris 7 (UPD7), Université de Technologie de Belfort-Montbeliard (UTBM)-Ecole Nationale Supérieure de Mécanique et des Microtechniques (ENSMM)-Université de Franche-Comté (UFC), Université Bourgogne Franche-Comté [COMUE] (UBFC)-Université Bourgogne Franche-Comté [COMUE] (UBFC)-Centre National de la Recherche Scientifique (CNRS), Université Paris Diderot - Paris 7 (UPD7)-Hôpital Robert Debré-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Hématologie pédiatrique, CHU Toulouse [Toulouse], CHU Saint-Etienne, Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Aix Marseille Université (AMU), Service de médecine de l'enfant et de l'adolescent, Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES), Université Sciences et Technologies - Bordeaux 1-CHU Bordeaux [Bordeaux], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Necker - Enfants Malades [AP-HP], Nutrition et Neurobiologie intégrée (NutriNeur0), Ecole nationale supérieure de chimie, biologie et physique-Institut Polytechnique de Bordeaux-Université Sciences et Technologies - Bordeaux 1-Institut National de la Recherche Agronomique (INRA)-Université Bordeaux Segalen - Bordeaux 2, Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Pitié-Salpêtrière [APHP], Université Joseph Fourier - Grenoble 1 (UJF)-Institut polytechnique de Grenoble - Grenoble Institute of Technology (Grenoble INP)-IMAG-Centre National de la Recherche Scientifique (CNRS)-Université Grenoble Alpes (UGA)-Université Joseph Fourier - Grenoble 1 (UJF)-Institut polytechnique de Grenoble - Grenoble Institute of Technology (Grenoble INP)-IMAG-Centre National de la Recherche Scientifique (CNRS)-Université Grenoble Alpes (UGA), Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Diderot - Paris 7 (UPD7)-Centre National de la Recherche Scientifique (CNRS), Institut de biologie structurale (IBS - UMR 5075), Institut de Recherche Interdisciplinaire de Grenoble (IRIG), Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Université Grenoble Alpes (UGA)-Centre National de la Recherche Scientifique (CNRS), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-CHU Toulouse [Toulouse]-Institut National de la Santé et de la Recherche Médicale (INSERM), Hôpital Sud, Interactions hôte-greffon-tumeur, ingénierie cellulaire et génique - UFC (UMR INSERM 1098) (HOTE GREFFON), Institut National de la Santé et de la Recherche Médicale (INSERM)-Etablissement français du sang [Bourgogne-France-Comté] (EFS [Bourgogne-France-Comté])-Université de Franche-Comté (UFC), CIC - Clermont Ferrand, Institut National de la Santé et de la Recherche Médicale (INSERM), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP), Université de Versailles Saint-Quentin-en-Yvelines (UVSQ)-Université Paris-Sud - Paris 11 (UP11)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Paul Brousse-Institut National de la Santé et de la Recherche Médicale (INSERM), Apoptose et Progression tumorale (CRCNA / Equipe 9), Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut de Recherche en Santé de l'Université de Nantes (IRS-UN), Service de médecine interne, Université Paris-Sud - Paris 11 (UP11)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Bicêtre, Centre Hospitalier Régional Universitaire de Brest (CHRU Brest)-Hôpital Morvan [Brest], Hôpital Claude Huriez, Université de Lille, Droit et Santé-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Université de Tours, Laboratoire Chrono-environnement - UFC (UMR 6249) (LCE), Université Bourgogne Franche-Comté [COMUE] (UBFC)-Centre National de la Recherche Scientifique (CNRS)-Université de Franche-Comté (UFC), Centre National de la Recherche Scientifique (CNRS)-Université de Limoges (UNILIM)-Génomique, Environnement, Immunité, Santé, Thérapeutique (GEIST FR CNRS 3503), Centre National de la Recherche Scientifique (CNRS)-Université de Montpellier (UM), Centre collaborateur de l'OMS Listeria - Biologie des Infections (CCOMS), Institut Pasteur [Paris]-Institut National de la Santé et de la Recherche Médicale (INSERM), Université Joseph Fourier - Grenoble 1 (UJF)-Institut polytechnique de Grenoble - Grenoble Institute of Technology (Grenoble INP)-Institut Polytechnique de Grenoble - Grenoble Institute of Technology-Centre National de la Recherche Scientifique (CNRS)-Université Grenoble Alpes (UGA), Université Paris Diderot - Paris 7 (UPD7)-CHU Saint Louis [APHP], McGill University, Université de Franche-Comté (UFC)-Centre National de la Recherche Scientifique (CNRS)-Ecole Nationale Supérieure de Mécanique et des Microtechniques (ENSMM)-Université de Technologie de Belfort-Montbeliard (UTBM), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Robert Debré-Université Paris Diderot - Paris 7 (UPD7), Centre de Recherche sur le Cancer Nantes-Angers (LUNAM), Université d'Angers (UA)-Université de Nantes (UN), Centre Hospitalier Universitaire d'Amiens (CHU), centre hospitalier universitaire amiens, CHU Bordeaux [Bordeaux]-Université Sciences et Technologies - Bordeaux 1, Université Joseph Fourier - Grenoble 1 (UJF)-Institut polytechnique de Grenoble - Grenoble Institute of Technology (Grenoble INP )-VetAgro Sup - Institut national d'enseignement supérieur et de recherche en alimentation, santé animale, sciences agronomiques et de l'environnement (VAS)-Centre National de la Recherche Scientifique (CNRS)-Université Joseph Fourier - Grenoble 1 (UJF)-Institut polytechnique de Grenoble - Grenoble Institute of Technology (Grenoble INP )-VetAgro Sup - Institut national d'enseignement supérieur et de recherche en alimentation, santé animale, sciences agronomiques et de l'environnement (VAS)-Centre National de la Recherche Scientifique (CNRS), and Institut National de la Santé et de la Recherche Médicale (INSERM)-Etablissement français du sang [Bourgogne-Franche-Comté] (EFS [Bourgogne-Franche-Comté])-Université de Franche-Comté (UFC)
Subjects: 0301 basic medicine, Primary Immunodeficiency Diseases, medicine.medical_treatment, Genetic counseling, Immunology, Genomics, Bioinformatics, Targeted therapy, 03 medical and health sciences, 0302 clinical medicine, Genotype, Humans, Immunology and Allergy, Medicine, Genetic Testing, Registries, Retrospective Studies, [SDV.BBM.BS]Life Sciences [q-bio]/Biochemistry, Molecular Biology/Structural Biology [q-bio.BM], business.industry, Common variable immunodeficiency, Retrospective cohort study, medicine.disease, Phenotype, 3. Good health, [SDV.BBM.BS]Life Sciences [q-bio]/Biochemistry, Molecular Biology/Biomolecules [q-bio.BM], 030104 developmental biology, Identification (biology), France, business, 030215 immunology
Abstract: International audience; To the Editor:Since the mid-1980s, continuous progress in genetics and genomics has accelerated the rapid identification of causative genetic variants leading to primary immunodeficiencies (PIDs; >300 genes),1 with the noticeable exception of B-cell disorders, such as common variable immunodeficiency (CVID). The identification of these mutations not only validates a clinical diagnosis but also is useful in several other respects (more accurate prognosis on phenotype/genotype correlation, targeted therapy, and genetic counseling). [...]
Published: 2019
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3. Growth deceleration in children treated with imatinib for chronic myeloid leukaemia

Author: Claire Galambrun, Fanny Rialland, Pascal Chastagner, Gérard Couillault, Joelle Guilhot, Françoise Mazingue, Christian Berthou, Nicolas Sirvent, Cécile Vérité, Virginie Gandemer, Frédéric Millot, Karima Yacouben, Tackwa Khalifeh, Yves Reguerre, André Baruchel, Thierry Leblanc, Patrick Lutz, Yves Bertrand, and Arnaud Petit
Subjects: Male, Cancer Research, Pediatrics, medicine.medical_specialty, Adolescent, Antineoplastic Agents, Chronic myeloid leukaemia, Piperazines, Body Mass Index, Growth velocity, Leukemia, Myelogenous, Chronic, BCR-ABL Positive, hemic and lymphatic diseases, Humans, Medicine, In patient, Child, neoplasms, Growth Disorders, Retrospective Studies, business.industry, Infant, Imatinib, Retrospective cohort study, medicine.disease, Body Height, Leukemia, Pyrimidines, Imatinib mesylate, Oncology, Child, Preschool, Benzamides, Imatinib Mesylate, Female, business, Body mass index, medicine.drug
Abstract: Purpose The aim is to study statural growth in a large cohort of children with chronic myeloid leukaemia (CML) treated with front-line imatinib. Methods Retrospective data from 81 children less than 18 years of age with CML identified in the French pediatric registry were analysed. Height was expressed as standard deviation score (SDS). Results A gradual decrease in height SDS was observed over time since starting imatinib. The height SDS was significantly lower 12 months and 24 months after the start of imatinib overall ( p −4 ) irrespective of gender and pubertal age. The height SDS was significantly ( p −4 ) lower 12 months after the start of imatinib in boys and girls, and in the prepubertal age group as well as in the postpubertal age group, respectively. A similar finding was observed in the subgroups of boys and girls starting imatinib at a prepubertal or postpubertal age. Loss in height SDS 12 months after the start of imatinib was of the same range in boys when compared to girls and in patients who started imatinib at a prepubertal age compared to those who started at a postpubertal age. Conclusion Growth velocity was altered during the first years of imatinib treatment in boys as well as in girls and in prepubertal age patients as well as in adolescents.
Published: 2014
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4. Les leucémies aiguës lymphoblastiques congénitales : expérience française entre 1990 et 2010

Author: Odile Minckes, Nicolas Sirvent, C. Vérité, Jacqueline Clavel, Martine Munzer, E. Plouvier, Virginie Gandemer, Guy Leverger, Y. Huguenin, Thierry Leblanc, X. Rialland, C. Thomas, Y Perel, G. Michel, C. Delvodere-Bécot, M. Granier, W. Abouh Chahla, Dominique Plantaz, Brigitte Nelken, Gérard Couillault, Yves Bertrand, Jean-Louis Stephan, and P. Blouin
Subjects: Oncology, Pediatrics, Perinatology and Child Health, Hematology
Abstract: Resume Propos Le but de notre etude est de decrire les leucemies aigues lymphoblastiques du 1 er mois de vie sur une base populationnelle exhaustive, d’en analyser les modalites de prise en charge et l’approche ethique. Methodes Nous avons effectue une etude retrospective multicentrique des cas de leucemie aigue lymphoblastique congenitale diagnostiques en France de 1990 a 2010, repertories dans le Registre national des hemopathies malignes de l’enfant et avons analyse leurs caracteristiques cliniques et biologiques, les modalites de traitement, l’evolution et les facteurs pronostiques. Resultats Vingt-deux enfants ont ete inclus, avec un âge median au diagnostic de 5,5 jours. Il est identifie un remaniement en 11q23 ou rearrangement MLL dans 17/22 cas. Le traitement a ete administre avec une ambition curative chez 14 patients ou centre sur une prise en charge palliative chez 7 patients. La survie globale a 2 ans est de 4,5 % ; un seul patient est survivant a long terme, avec de lourdes sequelles neurologiques. En analyse univariee, l’âge inferieur a huit jours au diagnostic, la corticoresistance et l’absence d’obtention de remission complete constituent des facteurs significatifs de mauvais pronostic. Conclusion Dans l’experience que nous rapportons, basee sur un enregistrement national exhaustif, les LAL congenitales apparaissent plus precoces et plus severes que dans les essais therapeutiques ou les nouveau-nes sont inclus de facon selective. Le pronostic oncologique extremement pejoratif conduit a inclure dans la strategie therapeutique la necessite ethique de preserver le confort de l’enfant et d’accompagner les processus d’attachement voire de deuil des parents et de la famille.
Published: 2014
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5. Development of primary early-onset colorectal cancers due to biallelic mutations of the FANCD1/BRCA2 gene

Author: Charles Coutant, Laurent Arnould, Christophe Philippe, Patrick Callier, Pierre Fumoleau, Myriam Bronner, Emmanuelle Ferrant, Sarab Lizard, Amandine Baurand, Jean-Louis Jouve, Laurence Faivre, Carole Chery, Gérard Couillault, Alexandra Delignette, Catherine Loustalot, Caroline Chapusot, Côme Lepage, Thierry Frebourg, Emilie Degrolard-Courcet, Laurent Martin, Francine Mugneret, Marie-Martine Padeano, Salima El Chehadeh, Séverine Guiu, Philippe Jonveaux, Fanny Coron, Joanna Sokolowska, Cyril Hatem, Service de Pathologie [CHU de Dijon], Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Nutrition-Génétique et Exposition aux Risques Environnementaux (NGERE), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lorraine (UL), Service de Génétique [CHRU Nancy], Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Service de chirurgie [Centre Georges-François Leclerc], Centre Régional de Lutte contre le cancer Georges-François Leclerc [Dijon] (UNICANCER/CRLCC-CGFL), UNICANCER-UNICANCER, Département d'oncologie médicale [Centre Georges-François Leclerc], Centre de génétique - Centre de référence des maladies rares, anomalies du développement et syndromes malformatifs (CHU de Dijon), Service d'Hépato-Gastro-Entérologie (CHU de Dijon), Polyclinique du Parc - Clinique Drevon [Dijon], Service d'Hématologie Clinique (CHU de Dijon), Service de pédiatrie (CHU de Dijon), Service de radiologie [Centre Georges-François Leclerc], Département de Biologie et pathologie des tumeurs [Centre Georges-François Leclerc], UNICANCER, Laboratoire de cytogénétique (CHU de Dijon), Génétique du cancer et des maladies neuropsychiatriques (GMFC), Université de Rouen Normandie (UNIROUEN), Normandie Université (NU)-Normandie Université (NU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de génétique [Rouen], CHU Rouen, Normandie Université (NU)-Normandie Université (NU)-Université de Rouen Normandie (UNIROUEN), and Normandie Université (NU)
Subjects: Adult, Biallelic Mutation, RNA Splicing, [SDV]Life Sciences [q-bio], DNA Mutational Analysis, Biology, medicine.disease_cause, Article, Frameshift mutation, Genetics, medicine, Humans, Missense mutation, Age of Onset, Gene, Alleles, Genetics (clinical), BRCA2 Protein, Mutation, Point mutation, Computational Biology, Chromosome Breakage, Pedigree, 3. Good health, Amino Acid Substitution, Female, RNA Splice Sites, Chromosome breakage, Colorectal Neoplasms
Abstract: International audience; Fanconi anaemia (FA) is characterized by progressive bone marrow failure, congenital anomalies, and predisposition to malignancy. In a minority of cases, FA results from biallelic FANCD1/BRCA2 mutations that are associated with early-onset leukaemia and solid tumours. Here, we describe the clinical and molecular features of a remarkable family presenting with multiple primary colorectal cancers (CRCs) without detectable mutations in genes involved in the Mendelian predisposition to CRCs. We unexpectedly identified, despite the absence of clinical cardinal features of FA, a biallelic mutation of the FANCD1/BRCA2 corresponding to a frameshift alteration (c.1845_1846delCT, p.Asn615Lysfs*6) and a missense mutation (c.7802A>G, p.Tyr2601Cys). The diagnosis of FA was confirmed by the chromosomal analysis of lymphocytes. Reverse transcriptase (RT)-PCR analysis revealed that the c.7802A>G BRCA2 variation was in fact a splicing mutation that creates an aberrant splicing donor site and results partly into an aberrant transcript encoding a truncated protein (p.Tyr2601Trpfs*46). The atypical FA phenotype observed within this family was probably explained by the residual amount of BRCA2 with the point mutation c.7802A>G in the patients harbouring the biallelic FANCD1/BRCA2 mutations. Although this report is based in a single family, it suggests that CRCs may be part of the tumour spectrum associated with FANCD1/BRCA2 biallelic mutations and that the presence of such mutations should be considered in families with CRCs, even in the absence of cardinal features of FA.
Published: 2013
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6. Prospective survey of indoor fungal contamination in hospital during a period of building construction

Author: V. Fourquenet, O. Vagner, C. L’Ollivier, H. Jury, Frédéric Dalle, Alain Bonnin, B. Cuisenier, J.-P. Besancenot, Serge Aho, Ingrid Lafon, C. Thibaut, Céline Calinon, Marc Sautour, Nathalie Sixt, Denis Caillot, Gérard Couillault, Microbiologie Environnementale, Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand ( CHU Dijon ), Laboratoire Interactions Muqueuses Agents Transmissibles ( LIMA ), Université de Bourgogne ( UB ), and Programme Hospitalier de recherche Clinique 2004 (PHRC) Délégation de la Recherche Clinique de Bourgogne, Dijon France
Subjects: Microbiology (medical), Veterinary medicine, Fungal contamination, Air Microbiology, Colony Count, Microbial, Microbiology, Bjerkandera adusta, [ SDV.MP ] Life Sciences [q-bio]/Microbiology and Parasitology, Patients' Rooms, Air treatment, medicine, Humans, Air Conditioning, Hospital Design and Construction, Prospective Studies, hospital, [ SDV.MP.MYC ] Life Sciences [q-bio]/Microbiology and Parasitology/Mycology, Mycosis, Infection Control, biology, fungus, Fungi, General Medicine, Fungi imperfecti, Contamination, biology.organism_classification, medicine.disease, Ventilation, Spore, Aspergillus, Infectious Diseases, Air Pollution, Indoor, nosocomial infection, Penicillium, air treatment
Abstract: International audience; An 18-month survey of indoor fungal contamination was conducted in one haematology unit during a period of construction work. Air was sampled with a portable Air System Impactor and surfaces with contact Sabouraud plates. During this survey the mean concentration of viable fungi in air was 4.2 cfu/m(3) and that for surfaces was 1.7 cfu/plate. At the beginning of construction work, there were increases in airborne fungal spores (from 3.0 to 9.8 cfu/m(3)) in the unit, but concentrations did not exceed 10 cfu/m(3) during the 18-month period. The most frequently recovered airborne fungi were Penicillium spp. (27-38%), Aspergillus spp. (25%) and Bjerkandera adusta, a basidiomycete identified with molecular tools (7-12%). Blastomycetes accounted for more than 50% of the fungal flora on surfaces. Investigating the impact of a new air-treatment system (mobile Plasmair units), there were significant reductions in fungal contamination for the Plasmer -treated rooms, and in these rooms we observed the same level of fungal load whether construction work was in progress or not.
Published: 2007
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7. Age and high-dose methotrexate are associated to clinical acute encephalopathy in FRALLE 93 trial for acute lymphoblastic leukemia in children

Author: M. D. Tabone, P Levy, J.-P. Vannier, Marie-Françoise Auclerc, G. Michel, Virginie Gandemer, F. Demeocq, Gérard Couillault, Guy Leverger, Thierry Leblanc, C. Schmitt, Judith Landman-Parker, Yves Perel, André Baruchel, M N Dufourg, Service d'hématologie-immunologie-oncologie pédiatrique [CHU Trousseau], Université Pierre et Marie Curie - Paris 6 (UPMC)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU), Bordeaux 2 Laboratoire de Psychologie EA «Santé et qualité de vie», Université Bordeaux Segalen - Bordeaux 2, Georgia Institute of Technology [Lorraine, France], Epidémiologie des maladies infectieuses et modélisation (ESIM), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM), Pediatric Hematology and Oncology, CHU Rouen, Normandie Université (NU)-Normandie Université (NU), Jet Propulsion Laboratory (JPL), NASA-California Institute of Technology (CALTECH), Service d'hématologie pédiatrique, Groupe Hospitalier Saint Louis - Lariboisière - Fernand Widal [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Diderot - Paris 7 (UPD7), Dufourg MN, Landman-Parker J, Michel G, Levy P, Couillault G, Gandemer V, Tabone MD, Demeocq F, Vannier JP, Leblanc T, Leverger G, Baruchel A., Auclerc MF, Schmitt C, Perel Y, Université Pierre et Marie Curie - Paris 6 (UPMC)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Université Paris Diderot - Paris 7 (UPD7), CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Diderot - Paris 7 (UPD7)-Groupe Hospitalier Saint Louis - Lariboisière - Fernand Widal [Paris], and Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)
Subjects: Male, MESH: Combined Modality Therapy, Cancer Research, MESH: Risk Assessment, Gastroenterology, MESH: Dose-Response Relationship, Drug, chemistry.chemical_compound, 0302 clinical medicine, Maintenance therapy, MESH: Child, Child, MESH: Antimetabolites, Antineoplastic, Acute leukemia, Brain Diseases, Metabolic, Age Factors, Hematology, Precursor Cell Lymphoblastic Leukemia-Lymphoma, Combined Modality Therapy, MESH: Infant, 3. Good health, MESH: Methotrexate, Oncology, Methylprednisolone, Child, Preschool, 030220 oncology & carcinogenesis, Antifolate, Female, medicine.drug, Antimetabolites, Antineoplastic, medicine.medical_specialty, Adolescent, Neurotoxins, Encephalopathy, MESH: Brain Diseases, Metabolic, Risk Assessment, 03 medical and health sciences, Acute lymphocytic leukemia, Internal medicine, medicine, Humans, MESH: Neurotoxins, MESH: Adolescent, MESH: Age Factors, MESH: Precursor Cell Lymphoblastic Leukemia-Lymphoma, MESH: Humans, Dose-Response Relationship, Drug, business.industry, MESH: Child, Preschool, Infant, medicine.disease, MESH: Male, Surgery, Methotrexate, chemistry, Cytarabine, [SDV.SPEE]Life Sciences [q-bio]/Santé publique et épidémiologie, business, MESH: Female, 030217 neurology & neurosurgery
Abstract: International audience; The objective of the study was to assess acute neurotoxicity associated with triple intrathecal therapy (TIT)+/-high-dose methotrexate (HD MTX) in children with acute lymphoblastic leukemia (ALL). 1395 children were enrolled on FRALLE 93 protocol from 1993 to 1999. Lower-risk group (LR, n=182) were randomized to weekly low-dose MTX at 25 mg/m(2)/week (LD MTX, n=81) or HD MTX at 1.5 g/m(2)/2 weeks x 6 (n=77). Intermediate-risk group (IR, n=672) were randomized to LD MTX (n=290) or HD MTX at 8 g/m(2)/2 weeks x 4 (n=316). Higher-risk group (HR, n=541) prednisone-responder patients received LD MTX and cranial radiotherapy. HR group steroid resistant cases were grafted (autologous or allogenic). TIT (MTX, cytarabine and methylprednisolone) was given every 2 weeks during 16-18 weeks and every 3 months during maintenance therapy in LR and IR patients. 52 patients (3.7%) developed neurotoxicity. Isolated seizures: n=15 (1.1%), peripheral and spinal neuropathy: n=17 (1.2%) and encephalopathy: n=20 (1.4%). Age >10 years was significantly associated with neurotoxicity (P=0.01) and use of HD MTX is associated with encephalopathy (P=0.03). Sequels are reported respectively in 60 and 33% of spinal neuropathy and encephalopathy cases. Current strategies tailoring risk of neurological sequels has to be defined.
Published: 2006
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8. Lymphocyte-Predominant Hodgkin’s Lymphoma in Children: Therapeutic Abstention After Initial Lymph Node Resection—A Study of the French Society of Pediatric Oncology

Author: Odile Oberlin, M.J. Terrier-Lacombe, Christine Edan, J P Vannier, Yves Bertrand, Annie Babin-Boilletot, B. Pellegrino, C. Lambilliote, Thierry Leblanc, C. Beard, Isabelle Thuret, Dominique Plantaz, Georges Delsol, Guy Leverger, Gérard Couillault, F. Demeocq, H. Pacquement, Yves Perel, Judith Landman-Parker, and Claudine Schmitt
Subjects: Male, Cancer Research, medicine.medical_specialty, Adolescent, medicine.medical_treatment, medicine, Humans, Prospective Studies, Stage (cooking), Child, Prospective cohort study, Chemotherapy, business.industry, Standard treatment, Remission Induction, medicine.disease, Hodgkin's lymphoma, Combined Modality Therapy, Hodgkin Disease, Survival Analysis, Lymphoma, Surgery, Treatment Outcome, Oncology, Child, Preschool, Lymph Node Excision, Female, Lymphadenectomy, Neoplasm Recurrence, Local, business, Adenectomy
Abstract: Purpose: To clarify treatment strategy for lymphocyte-predominant Hodgkin’s lymphoma (LPHL), the French Society of Pediatric Oncology initiated a prospective, nonrandomized study in 1988. Patients received either standard treatment for Hodgkin’s lymphoma or were not treated beyond initial adenectomy. Patients and Methods: From 1988 to 1998, 27 patients were available for study. Twenty-four patients were male, and median age was 10 years (range, 4 to 16 years). Twenty-two, two, and three patients had stage I, II, and III disease, respectively. Thirteen patients (stage I, n = 11; stage III, n = 2) received no further treatment after initial surgical adenectomy (SA). Fourteen patients received combined treatment (CT; n = 10), involved-field radiotherapy alone (n = 1), or chemotherapy alone (n = 3). The two groups were comparable for clinical status, treatment, and follow-up. Results: Twenty-three of 27 patients achieved complete remission (CR). With a median follow-up time of 70 months (range, 32 to 214 months), overall survival to date is 100%, and overall event-free survival (EFS) is 69% ± 10% (SA, 42% ± 16%; CT, 90% ± 8.6%; P < .04). If we considered only the patients in CR after initial surgery (n = 12), EFS was no longer significantly different between the two groups. Patients with residual mass after initial surgery (n = 15) had worse EFS if they did not receive complementary treatment (P < .05). Conclusion: Although based on a small number of patients, our study showed that (1) no further therapy is a valid therapeutic approach in LPHL patient in CR after initial lymph node resection, and (2) complementary treatment diminishes relapse frequency but has no impact on survival.
Published: 2003
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9. Autoimmune and inflammatory manifestations occur frequently in patients with primary immunodeficiencies

Author: Alain Fischer, Johan Provot, Jean-Philippe Jais, Alexandre Alcais, Nizar Mahlaoui, Daniel Adoue, Nathalie Aladjidi, Zahir Amoura, Philippe Arlet, Corinne Armari-Alla, Brigitte Bader-Meunier, Vincent Barlogis, Sophie Bayart, Beatrice Beaurain, Yves Bertrand, Boris Bienvenu, Stéphane Blanche, Damien Bodet, Bernard Bonnotte, Raphaël Borie, Patrick Boutard, Claire Briandet, Jean-Paul Brion, Carolina Brito, Jacques Brouard, Emilie Catherinot, Olivia Chandesris, Sarah Cohen-Beaussant, Hélène Coignard-Biehler, Laurence Costes, Louis-Jean Couderc, Gérard Couillault, Virginie Courteille, Elodie Curlier, Geneviève de Saint Basile, François Demeocq, Nathalie de Vergnes, Catherine Devoldere, Anne Deville, Jean Donadieu, Eric Dore, Fabienne Dulieu, Isabelle Durieu, Christine Edan, Natacha Entz Werle, Claire Fieschi, Fanny Fouyssac, Pierre Frange, Vincent Gajdos, Lionel Galicier, Virginie Gandemer, Martine Gardembas, Catherine Gaud, Bernard Grosbois, Gaelle Guillerm, Eric Hachulla, Mohamed Hamidou, Sébastien Héritier, Olivier Hermine, Cyrille Hoarau, Bruno Hoen, Arnaud Hot, Sébastien Humbert, Arnaud Jaccard, Serge Jacquot, Rolland Jaussaud, Pierre-Yves Jeandel, Eric Jeziorski, Kamila Kebaili, Anne-Sophie Korganow, Philippe Labrune, Olivier Lambotte, Fanny Lanternier, Claire Larroche, Alain Le Quellec, Emmanuelle Le Moigne, Vincent Le Moing, Yvon Lebranchu, Marc Lecuit, Guillaume Lefevre, Richard Lemal, Philippe Le Moine, Valérie Li Thiao Te, Olivier Lortholary, Patrick Lutz, Aude Magerus-Chatinet, Marion Malphettes, Aude Marie-Cardine, Nicolas Martin Silva, Agathe Masseau, Christian Massot, Françoise Mazingue, Etienne Merlin, Gérard Michel, Frédéric Millot, Odile Minckes, Béatrice Monlibert, Fabrice Monpoux, Despina Moshous, Luc Mouthon, Martine Munzer, Bénédicte Neven, Raphaëlle Nove-Josserand, Eric Oksenhendler, Marie Ouachée-Chardin, Anne Pagnier, Jean-Louis Pasquali, Marlène Pasquet, Isabelle Pellier, Yves Perel, Antoinette Perlat, Capucine Picard, Christophe Piguet, Dominique Plantaz, Pierre Quartier, Frédéric Rieux-Laucat, Pascal Roblot, Pierre-Marie Roger, Pierre-Simon Rohrlich, Bruno Royer, Valéry Salle, Françoise Sarrot-Reynauld, Amélie Servettaz, Jean-Louis Stephan, Nicolas Schleinitz, Felipe Suarez, Laure Swiader, Sophie Taque, Caroline Thomas, Olivier Tournilhac, Caroline Thumerelle, Jean-Pierre Vannier, Jean-François Viallard, Centre de Référence Déficits Immunitaires Héréditaires (CEREDIH), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service d'immuno-hématologie pédiatrique [CHU Necker], Imagine - Institut des maladies génétiques (IMAGINE - U1163), Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM), Collège de France - Chaire Médecine expérimentale (A. Fischer), Collège de France (CdF (institution)), Centre de Recherche des Cordeliers (CRC), Université Pierre et Marie Curie - Paris 6 (UPMC)-École Pratique des Hautes Études (EPHE), Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL)-Université Paris Diderot - Paris 7 (UPD7)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Necker - Enfants Malades [AP-HP], Centre National de la Recherche Scientifique (CNRS)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM), Collège de France (CdF), Université Paris Diderot - Paris 7 (UPD7)-École pratique des hautes études (EPHE)-Université Pierre et Marie Curie - Paris 6 (UPMC)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM), Chaire Médecine expérimentale (A. Fischer), Université Paris Diderot - Paris 7 (UPD7)-École pratique des hautes études (EPHE), and Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL)-Université Pierre et Marie Curie - Paris 6 (UPMC)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)
Subjects: Adult, Male, Risk, 0301 basic medicine, Primary immunodeficiencies, Adolescent, T-Lymphocytes, Immunology, Population, Context (language use), medicine.disease_cause, Inflammatory bowel disease, Autoimmunity, Young Adult, 03 medical and health sciences, Prevalence, medicine, Humans, Immunology and Allergy, Age of Onset, [SDV.IMM.ALL]Life Sciences [q-bio]/Immunology/Allergology, Child, education, Aged, Retrospective Studies, Aged, 80 and over, Autoimmune disease, Purpura, Thrombocytopenic, Idiopathic, education.field_of_study, business.industry, Common variable immunodeficiency, Autoimmune Cytopenia, autoimmunity, Immunologic Deficiency Syndromes, Infant, Middle Aged, Inflammatory Bowel Diseases, Prognosis, medicine.disease, Survival Analysis, 3. Good health, 030104 developmental biology, inflammation, Child, Preschool, Primary immunodeficiency, Female, France, business
Abstract: International audience; BACKGROUND:Primary immunodeficiencies (PIDs) are inherited diseases associated with a considerable increase in susceptibility to infections. It is known that PIDs can also predispose to cancer and immune diseases, including allergy, autoimmunity, and inflammation.OBJECTIVE:We aimed at determining the incidence of autoimmunity and inflammation in patients with PIDs.METHODS:We have retrospectively screened 2183 consecutive cases of PID in the Centre de Référence Déficits Immunitaires Héréditaires registry (CEREDIH; the French national PID registry) for the occurrence of autoimmunity and inflammation.RESULTS:One or more autoimmune and inflammatory complications were noted in 26.2% of patients, with a risk of onset throughout the patient's lifetime. The risk of autoimmune cytopenia was at least 120 times higher than in the general population, the risk of inflammatory bowel disease in children was 80 times higher, and the risk of other autoimmune manifestations was approximately 10 times higher. Remarkably, all types of PIDs were associated with a risk of autoimmune and inflammatory complications, although the greatest risk was associated with T-cell PIDs and common variable immunodeficiency. The occurrence of autoimmune disease is a negative prognostic factor for survival.CONCLUSIONS:Our results provide the basis for a detailed prospective evaluation of autoimmunity and inflammation in the context of PIDs, with a view to accurately assessing these risks and describing the possible effect of medical intervention.
Published: 2017
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10. Human RTEL1 deficiency causes Hoyeraal-Hreidarsson syndrome with short telomeres and genome instability

Author: Tangui Le Guen, Gérard Couillault, Laetitia Gaillard, Jean Soulier, Capucine Picard, Jean-Pierre de Villartay, Fabien Touzot, Patrick Nitschke, Isabelle Callebaut, Arturo Londoño-Vallejo, Michael Schertzer, Nada Jabado, Alain Fischer, Mylène Perderiset, Wassila Carpentier, Patrick Revy, Laurent Jullien, Thomas, Danielle, Centre de recherche en Biologie Cellulaire (CRBM), Université de Montpellier (UM)-Centre National de la Recherche Scientifique (CNRS), Imagine - Institut des maladies génétiques (IMAGINE - U1163), Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service d'immuno-hématologie pédiatrique [CHU Necker], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Telomeres and Cancer Laboratory, Institut Curie [Paris], Plateforme Post-génomique de la Pitié-Salpêtrière (P3S), UMS omique (OMIQUE), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de pédiatrie (CHU de Dijon), Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Pathologie cellulaire : aspects moléculaires et viraux / Pathologie et Virologie Moléculaire, Institut Universitaire d'Hématologie (IUH), Université Paris Diderot - Paris 7 (UPD7)-Université Paris Diderot - Paris 7 (UPD7)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Groupe Hospitalier Saint Louis - Lariboisière - Fernand Widal [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Centre National de la Recherche Scientifique (CNRS), Collège de France - Chaire Médecine expérimentale (A. Fischer), Collège de France (CdF (institution)), Institut de minéralogie et de physique des milieux condensés (IMPMC), Université Pierre et Marie Curie - Paris 6 (UPMC)-Université Paris Diderot - Paris 7 (UPD7)-Institut de Physique du Globe de Paris (IPG Paris)-Centre National de la Recherche Scientifique (CNRS), Department of Human Genetics , Department of Experimental Medicine, Radboud University Medical Center [Nijmegen], Université Montpellier 1 (UM1)-Université Montpellier 2 - Sciences et Techniques (UM2)-Université de Montpellier (UM)-Centre National de la Recherche Scientifique (CNRS), Chaire Médecine expérimentale (A. Fischer), Université Pierre et Marie Curie - Paris 6 (UPMC)-IPG PARIS-Université Paris Diderot - Paris 7 (UPD7)-Centre National de la Recherche Scientifique (CNRS), Centre de recherches de biochimie macromoléculaire ( CRBM ), Université Montpellier 1 ( UM1 ) -Université Montpellier 2 - Sciences et Techniques ( UM2 ) -IFR122-Centre National de la Recherche Scientifique ( CNRS ), Imagine - Institut des maladies génétiques ( IMAGINE - U1163 ), Université Paris Descartes - Paris 5 ( UPD5 ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Centre National de la Recherche Scientifique ( CNRS ), Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Necker - Enfants Malades [AP-HP], Plateforme Post-génomique de la Pitié-Salpêtrière ( P3S ), UMS omique ( OMIQUE ), Université Pierre et Marie Curie - Paris 6 ( UPMC ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Université Pierre et Marie Curie - Paris 6 ( UPMC ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), Service de pediatrie, Groupe Hospitalier Saint Louis - Lariboisière - Fernand Widal [Paris]-Université Paris Diderot - Paris 7 ( UPD7 ) -Institut Universitaire d'Hématologie ( IUH ), Université Paris Diderot - Paris 7 ( UPD7 ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Centre National de la Recherche Scientifique ( CNRS ), Institut de minéralogie et de physique des milieux condensés ( IMPMC ), Université Pierre et Marie Curie - Paris 6 ( UPMC ) -IPG PARIS-Université Paris Diderot - Paris 7 ( UPD7 ) -Centre National de la Recherche Scientifique ( CNRS ), and Department of Human Genetics
Subjects: Genome instability, DNA Replication, Male, DNA damage, DNA repair, Genetic Linkage, Hoyeraal-Hreidarsson syndrome, Biology, medicine.disease_cause, Dyskeratosis Congenita, Genomic Instability, 03 medical and health sciences, 0302 clinical medicine, Intellectual Disability, Genetics, medicine, [SDV.BBM] Life Sciences [q-bio]/Biochemistry, Molecular Biology, Humans, [SDV.BBM]Life Sciences [q-bio]/Biochemistry, Molecular Biology, Exome, Molecular Biology, [ SDV.BBM ] Life Sciences [q-bio]/Biochemistry, Molecular Biology, Telomerase, Genetics (clinical), Cells, Cultured, Telomere Shortening, 030304 developmental biology, 0303 health sciences, Mutation, Fetal Growth Retardation, Sequence Analysis, RNA, DNA replication, DNA Helicases, Infant, Telomere Homeostasis, General Medicine, Telomere, medicine.disease, 3. Good health, 030220 oncology & carcinogenesis, Child, Preschool, Microcephaly, Female, Sequence Alignment, Dyskeratosis congenita, DNA Damage
Abstract: International audience; Hoyeraal-Hreidarsson syndrome (HHS), a severe variant of dyskeratosis congenita (DC), is characterized by early onset bone marrowfailure, immunodeficiency and developmental defects. Several factors involved in telomere length maintenance and/or protection are defective in HHS/DC, underlining the relationship between telomeredysfunctionandthese diseases.Bycombining whole-genomelinkage analysisandexomesequencing,we identified compound heterozygous RTEL1 (regulator of telomere elongation helicase 1) mutations in three patients with HHS from two unrelated families. RTEL1 is a DNA helicase that participates in DNA replication, DNA repair and telomere integrity. We show that, in addition to short telomeres, RTEL1-deficient cells from patients exhibit hallmarks of genome instability, including spontaneous DNA damage, anaphase bridges and telomeric aberrations. Collectively, these results identify RTEL1 as a novel HHS-causing gene and highlight its role as a genomic caretaker in humans.
Published: 2013
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11. Efficacy of vinblastine in central nervous system Langerhans cell histiocytosis: a nationwide retrospective study

Author: Karima Mokhtari, Mohamed Barkaoui, Marie-Anne Barthez, Catherine Garel, Dominique Plantaz, Anne Lambilliotte, Julien Haroche, Gérard Couillault, Jean Donadieu, Nadine Martin-Duverneuil, Khê Hoang-Xuan, Olivier Aumaître, Alain Robert, Perrine Marec Bérard, Ahmed Idbaih, Patrick Lutz, Anne-Sophie Defachelles, Jean Sibilia, Hubert Ducou Le Pointe, Jean-Loup Pennaforte, Ghislain Nokam Talom, Corinne Armari-Alla, Martine Munzer, Martine Gardembas, T Généreau, Judith Landman Parker, Sophie Ng Wing Tin, Renato Fior, Michel Polak, and Maria Ribeiro
Subjects: Adult, Central Nervous System, Male, medicine.medical_specialty, Adolescent, lcsh:Medicine, Vinblastine, Gastroenterology, Young Adult, Langerhans cell histiocytosis, Central Nervous System Diseases, Internal medicine, medicine, Humans, Genetics(clinical), Pharmacology (medical), Child, Adverse effect, Genetics (clinical), Retrospective Studies, Medicine(all), business.industry, Research, Standard treatment, lcsh:R, Infant, Retrospective cohort study, General Medicine, Middle Aged, medicine.disease, Antineoplastic Agents, Phytogenic, Clinical trial, Histiocytosis, Langerhans-Cell, Histiocytosis, Treatment Outcome, Child, Preschool, Concomitant, Immunology, Female, Steroids, business, medicine.drug
Abstract: Background Vinblastine (VBL) is the standard treatment for systemic Langerhans cell histiocytosis (LCH), but little is known about its efficacy in central nervous system (CNS) mass lesions. Methods A retrospective chart review was conducted. Twenty patients from the French LCH Study Group register met the inclusion criteria. In brief, they had CNS mass lesions, had been treated with VBL, and were evaluable for radiologic response. Results The median age at diagnosis of LCH was 11.5 years (range: 1-50). Intravenous VBL 6 mg/m2 was given in a 6-week induction treatment, followed by a maintenance treatment. The median total duration was 12 months (range: 3-30). Eleven patients received steroids concomitantly. Fifteen patients achieved an objective response; five had a complete response (CR: 25%), ten had a partial response (PR: 50%), four had stable disease (SD: 20%) and one patient progressed (PD: 5%). Of interest, four out of the six patients who received VBL without concomitant steroids achieved an objective response. With a median follow-up of 6.8 years, the 5-year event-free and overall survival was 61% and 84%, respectively. VBL was well-tolerated and there were no patient withdrawals due to adverse events. Conclusion VBL, with or without steroids, could potentially be a useful therapeutic option in LCH with CNS mass lesions, especially for those with inoperable lesions or multiple lesions. Prospective clinical trials are warranted for the evaluation of VBL in this indication.
Published: 2011
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12. Imatinib is effective in children with previously untreated chronic myelogenous leukemia in early chronic phase: results of the French national phase IV trial

Author: Arnaud Petit, Christian Berthou, Francoise Mechinaud, Pierre Bordigoni, Claire Galambrun, Jean-Michel Cayuela, Patrick Lutz, Karima Yacouben, Joelle Guilhot, Christine Edan, André Baruchel, Thierry Leblanc, Françoise Mazingue, Yves Bertrand, Gérard Couillault, Frédéric Millot, Cécile Vérité, Frédéric Bernard, Françoise Guilhot, and Yves Reguerre
Subjects: Cancer Research, medicine.medical_specialty, Adolescent, Drug-Related Side Effects and Adverse Reactions, Antineoplastic Agents, Neutropenia, Disease-Free Survival, Piperazines, hemic and lymphatic diseases, Internal medicine, Leukemia, Myelogenous, Chronic, BCR-ABL Positive, Medicine, Humans, Prospective Studies, Adverse effect, Child, Survival rate, Dose-Response Relationship, Drug, business.industry, Infant, Imatinib, medicine.disease, Survival Analysis, Surgery, Clinical trial, Imatinib mesylate, Pyrimidines, Oncology, Child, Preschool, Benzamides, Imatinib Mesylate, France, business, Complete Hematologic Response, Chronic myelogenous leukemia, medicine.drug
Abstract: Purpose Imatinib is the standard of care in adults with chronic myeloid leukemia (CML) in chronic phase (CP). Only a few studies to assess efficacy in children have been performed. We report on the results of the French prospective trial (ClinicalTrials.gov identifier NCT00845221) conducted in children and adolescents with newly diagnosed CML in CP. Patients and Methods A total of 44 patients from age 10 months to 17 years with newly diagnosed CML in CP received daily imatinib 260 mg/m2. Progression-free survival, responses, and tolerance were evaluated. Results With a median follow-up times of 31 months (range, 11 to 64 months), the estimated progression-free survival rate at 36 months was 98% (95% CI, 85% to 100%). A complete hematologic response was achieved in 98% of the patients. The rates of complete cytogenetic response (CCyR) and major molecular response (MMR) were 61% and 31% at 12 months, respectively. During follow-up, CCyR and MMR were achieved in 36 children (77%) and 25 children (57%), respectively. Overall, 30% of the patients discontinued imatinib, mainly because of unsatisfactory response. The most common adverse events were neutropenia and musculoskeletal events. Conclusion Imatinib is effective in children with CML in CP with response rates similar to rates reported in adults. The adverse effects are acceptable, but longer follow-up studies are required to fully assess the long-term impact.
Published: 2011

13. Profiles and seasonal distribution of airborne fungi in indoor and outdoor environments at a French hospital

Author: B. Cuisenier, Frédéric Dalle, Nathalie Sixt, Alain Bonnin, Gérard Couillault, Claire Cachia, Stéphanie Valvin, O. Vagner, Vitalie Fourquenet, Marc Sautour, Céline Calinon, Denis Caillot, Kusum Paul, Alix Maurel, C. L’Ollivier, Serge Aho, Laboratoire Interactions Muqueuses Agents Transmissibles ( LIMA ), Université de Bourgogne ( UB ), CHU Dijon, Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand ( CHU Dijon ), Mort cellulaire et cancer, Université de Bourgogne ( UB ) -IFR100 - Structure fédérative de recherche Santé-STIC-Institut National de la Santé et de la Recherche Médicale ( INSERM ), Santé - STIC, Université de Bourgogne ( UB ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), Laboratoire Interactions Muqueuses Agents Transmissibles (LIMA), Université de Bourgogne (UB), Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Université de Bourgogne (UB)-IFR100 - Structure fédérative de recherche Santé-STIC-Institut National de la Santé et de la Recherche Médicale (INSERM), and Université de Bourgogne (UB)-Institut National de la Santé et de la Recherche Médicale (INSERM)
Subjects: Veterinary medicine, Environmental Engineering, Seasonal distribution, Air Microbiology, 010501 environmental sciences, 01 natural sciences, 03 medical and health sciences, Bjerkandera adusta, [ SDV.MP ] Life Sciences [q-bio]/Microbiology and Parasitology, medicine, Environmental Chemistry, Hospital Design and Construction, Waste Management and Disposal, Air quality index, Airborne fungi Outdoor and indoor air Hospital Haematology units Seasonal variations Aspergillus, 0105 earth and related environmental sciences, 0303 health sciences, Aspergillus, biology, 030306 microbiology, Ecology, Fungi, Fungi imperfecti, Seasonality, biology.organism_classification, medicine.disease, Pollution, [SDV.MP]Life Sciences [q-bio]/Microbiology and Parasitology, Air Pollution, Indoor, Penicillium, Particulate Matter, France, Seasons, Environmental Monitoring, Cladosporium
Abstract: International audience; A one-year prospective survey of fungal air contamination was conducted in outdoor air and inside two haematological units of a French hospital. Air was sampled with a portable Air System Impactor. During this period of survey, the mean viable fungal load was 122.1 cfu/m(3) in outdoor air samples, and 4.1 and 3.9 cfu/m(3) in samples from adult and pediatric haematology units, respectively. In outdoor samples, Cladosporium was the dominant genus (55%) while in the clinical units, Penicillium sp. (23 to 25%), Aspergillus sp. (15 to 23%) and Bjerkandera adusta (11 to 13%) were the most frequently recovered airborne fungi. The outdoor fungal load was far higher in autumn (168 cfu/m(3)), spring (110 cfu/m(3)) and summer (138 cfu/m(3)) than in winter (49 cfu/m(3)). In indoor air, fungal concentrations were significantly lower in winter (2.7 to 3.1 cfu/m(3)) than in summer (4.2 to 5.0 cfu/m(3)) in both haematology units. In the outdoor environment, Penicillium sp. and Aspergillus sp. were more abundant in winter while the levels of Cladosporium were lowest during this season. In the haematological units, the presence of Aspergillus sp. was stable during the year (close to 20%), Bjerkandera sp. was particularly abundant in winter (close to 30%); levels of Penicillium sp. were highest in autumn while levels of Cladosporium sp. were highest in spring and summer.
Published: 2009
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14. Spontaneous macrophage colony-forming units in juvenile myelomonocytic leukaemia

Author: François Girodon, Emilie Bergoin, Marc Maynadié, Marie-Hélène Deny, and Gérard Couillault
Subjects: Colony-forming unit, medicine.medical_specialty, Cytopenia, Hematology, Juvenile myelomonocytic leukemia, Immature Granulocyte, business.industry, Macrophages, Infant, Leukemia, Myelomonocytic, Chronic, medicine.disease, Andrology, medicine.anatomical_structure, Internal medicine, White blood cell, Immunology, medicine, Neoplastic Stem Cells, Macrophage, Humans, Platelet, Female, business, Tumor Stem Cell Assay
Abstract: An 8-month-old girl was referred to the hospital for cytopenia: the blood count showed haemoglobin 86 g/l, white blood cell count (WBC) 10.9 · 10/l with 9% neutrophils, 60% lymphocytes, 2% activated lymphocytes, 1% eosinophils, 23% monocytes and 5% immature granulocytes without blast cells. The platelet count was 28 · 10/l. The bone marrow aspirate was poor and did not show marked cytological abnormalities; few megakaryocytes were observed. The blood count recovered over several weeks, but the monocyte count remained above 2 · 10/l. When she was 1 year old, splenomegaly was noted, associated with an abnormal blood cell count: Hb 94 g/l, WBC 30AE3 · 10/l with 27% neutrophils, 4% eosinophils, 3% basophils, 36% lymphocytes, 22% monocytes and 8% immature granulocytes. The platelet count was 136 · 10/l. There was a moderate increase in haemoglobin F (9%) and the karyotype, analysed twice, was normal. A bone marrow aspirate showed dysplastic features mainly of megakaryocytes and granulocytes, associated with a high monocyte count (22%). A stem cell culture performed in a serum-free medium based on collagen (Stemcell Technologies, France) showed endogenous macrophage colony-forming units (CFU-M) growth: on day 14, collagen gels were harvested onto glass slides, dried and stained with MayGrunwald-Giemsa then observed microscopically (figures). This spontaneous CFU-M growth in vitro is a specific feature of juvenile myelomonocytic leukaemia and was important in confirming the diagnosis in this patient. Bone marrow transplantation was carried out successfully when the infant was 18 months old.
Published: 2007

15. Hypereosinophilia in acute B-lineage lymphoblastic leukaemia

Author: Gérard Couillault, François Girodon, Sandrine-Anne Martha, Emilie Bergoin, Paule-Marie Carli, Marc Maynadié, Francine Mugneret, and Bernardine Favre
Subjects: medicine.medical_specialty, Lineage (genetic), Adolescent, Hypereosinophilia, Translocation, Genetic, Immunophenotyping, Internal medicine, Acute lymphocytic leukemia, Eosinophilia, medicine, Humans, In Situ Hybridization, Fluorescence, Chromosomes, Human, Pair 14, Hematology, medicine.diagnostic_test, business.industry, Bone Marrow Examination, medicine.disease, Burkitt Lymphoma, Bone marrow examination, Immunology, Cytogenetic Analysis, Lymphoblastic leukaemia, Chromosomes, Human, Pair 5, Female, medicine.symptom, business
Published: 2005

16. 'Agranular CD4+ CD56+ hematodermic neoplasm' (blastic NK-cell lymphoma) originates from a population of CD56+ precursor cells related to plasmacytoid monocytes

Author: Charles R. Maliszewski, Anne de Muret, Sophie Dalac, Marc Maynadié, Laurent J. Galibert, Tony Petrella, Michael R. Comeau, Anne Durlach, and Gérard Couillault
Subjects: Adult, Male, Pathology, medicine.medical_specialty, Cell type, Skin Neoplasms, T cell, Sialoglycoproteins, Population, Interleukin-3 Receptor alpha Subunit, Antigens, Differentiation, Myelomonocytic, Biology, Monocytes, Pathology and Forensic Medicine, Natural killer cell, Antigens, CD, medicine, Neoplasm, Humans, education, Child, Aged, Aged, 80 and over, education.field_of_study, Leukosialin, CD68, hemic and immune systems, Blastic NK cell lymphoma, HLA-DR Antigens, Middle Aged, medicine.disease, CD56 Antigen, Receptors, Interleukin-3, Lymphoma, Lymphoma, T-Cell, Cutaneous, Killer Cells, Natural, stomatognathic diseases, medicine.anatomical_structure, Phenotype, CD4 Antigens, Surgery, Female, Anatomy
Abstract: In 1999, we reported seven cases of an unusual hematologic malignancy with primary cutaneous presentation that appeared as a distinct clinicopathologic entity characterized by medium-sized tumor cells with a peculiar CD3- CD4+ CD56+ CD43+ HLA-DR+ cell surface phenotype. Because the origin of tumor cells was not clear and they exhibited a nonlineage-specific phenotype, we hypothesized that such tumors likely originated from hematologic-myeloid precursor cells and were tentatively assigned the designation "agranular CD4+ CD56+ hematodermic neoplasms." In the present study we report 14 cases (seven already reported and seven additional cases) of these tumors, and simultaneously we present now a rare population of cells that we have identified in the peripheral blood of healthy volunteers treated with Flt3 ligand. These cells express all the characteristic markers of CD4+ CD56+ hematodermic neoplasms. This population appears to be related to plasmacytoid monocytes because they also expressed CD68 and bright levels of CD123. To confirm the relationship between these normal cells and CD4+ CD56+ hematodermic neoplasms, we conducted an extensive comparative phenotypic study. Results show that these two cell types are indeed related because they share many phenotypic features, including the presence of CD4, CD56, CD43, CD68, and HLA-DR and the absence of other T, B, NK, or myelomonocytic markers. More importantly, we found that the bright expression of CD123 by immunohistochemistry is a distinctive characteristic of CD4+ CD56+ hematodermic neoplasms because all (n = 14) cases expressed this marker, whereas only two specimens in a control panel comprising 30 samples of related tumors expressed comparable levels of CD123. We therefore propose that oncogenic transformation of NCAM-expressing plasmacytoid monocyte-like cells may lead to "agranular CD4+ CD56+ hematodermic neoplasm."
Published: 2002

17. SFCE CO-09 - Syndrome CMMR-D : description clinique dans une série française

Author: Martine Muleris, Laurence Brugières, C. Colas, Sophie Lejeune, Gérard Couillault, Jean-Pierre Fricker, N. Entz-Werle, C. Charpy, Olivier Caron, Q. Wang, Odile Cabaret, G Sebille, Franck Bourdeaut, Thierry Frebourg, Michèle Mathieu-Dramard, Noémie Lavoine, Dominique Leroux, R. Guimbaud, and Guy Leverger
Subjects: Pediatrics, Perinatology and Child Health
Abstract: Objectif decrire les caracteristiques cliniques des patients francais atteints de CMMR-D. Sujet / materiels et methodes le syndrome CMMR-D est un syndrome de predisposition aux cancers de l’enfant du a une mutation biallelique des genes MMR. Il est meconnu des onco-pediatres et probablement sous-diagnostique. Une revue a ete menee a partir des dossiers cliniques des cas de CMMR-D diagnostiques par des laboratoires de genetique en France de 1999 a 2013. Resultats principaux 28 patients ont developpe 60 cancers, dont 17 hemopathies, 19 tumeurs cerebrales, 21 cancers du spectre du syndrome de Lynch, et 3 sarcomes. 19/28 patients ont developpe plusieurs cancers. L’âge median au diagnostic de premiere tumeur etait 7 ans [1,23–33,53]. La survie globale a 5 ans etait de 56% apres le premier cancer. 40% des deces etaient lies a l’evolution de la premiere tumeur. 21/28 patients presentaient des taches cafe-au-lait ou des macules depigmentees. L’histoire familiale evoquait un syndrome de Lynch dans 25% des familles, une consanguinite dans 1/3 des cas. Conclusion le syndrome CMMR-D predispose a la survenue de cancers precoces et au pronostic sombre. Son diagnostic est difficile mais indispensable pour mettre en place une surveillance appropriee chez les patients et leurs apparentes.
Published: 2014
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18. Acute Myeloid Leukemia (AML) in Children: Results of the French Trials

Author: B. Nelken, André Baruchel, G. Michel, Gérard Couillault, I. Thuret, G. Schaison, E. Le Gall, Judith Landman-Parker, Pierre Bordigoni, Thierry Leblanc, Francoise Mechinaud, Yves Perel, A. Auvrignon, J. P. Vannier, Jean-Louis Stephan, Guy Leverger, and H. Esperou
Subjects: Oncology, Down syndrome, medicine.medical_specialty, Bone marrow transplantation, business.industry, Pediatric acute myeloid leukemia, Induction chemotherapy, Myeloid leukemia, medicine.disease, Internal medicine, Induction therapy, medicine, business, Childhood AML
Abstract: During the past ten years major progress has been made in the treatment of childhood AML with the introduction of intensified induction therapy, followed by an aggressive post induction chemotherapy or an allogenic bone marrow transplantation.
Published: 2001
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19. Decreased Activity of Clotting Factor V During ALL Consolidation Treatment with Mercaptopurine in a Child

Author: Claire Vignand, Gérard Couillault, Genevieve Jolimoy, Fabienne Dutrillaux, and Catherine Sgro
Subjects: Clotting factor, medicine.medical_specialty, Endocrinology, Consolidation (soil), business.industry, Internal medicine, medicine, Pharmacology (medical), business, Mercaptopurine, medicine.drug
Published: 2006
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20. Improved management of invasive pulmonary aspergillosis in neutropenic patients using early thoracic computed tomographic scan and surgery

Author: B. Cuisenier, Denis Caillot, F Piard, Tony Petrella, Alain Bonnin, A. Bernard, Christine M. Durand, J.-F. Couaillier, Eric Solary, H. Guy, M. Dumas, Olivier Casasnovas, and Gérard Couillault
Subjects: Male, Cancer Research, medicine.medical_specialty, Antigens, Fungal, Neutropenia, Chest pain, Aspergillosis, Bronchoalveolar Lavage, Medicine, Humans, Mycosis, Retrospective Studies, Lung, medicine.diagnostic_test, Lung Diseases, Fungal, business.industry, Respiratory disease, Retrospective cohort study, Middle Aged, medicine.disease, Prognosis, Combined Modality Therapy, Surgery, medicine.anatomical_structure, Bronchoalveolar lavage, Aspergillus, Oncology, Female, medicine.symptom, business, Tomography, X-Ray Computed
Abstract: PURPOSE The prognosis of invasive pulmonary aspergillosis (IPA) occurring in neutropenic patients remains poor. We studied whether new strategies for early diagnosis could improve outcome in these patients. PATIENTS AND METHODS Twenty-three histologically proven and 14 highly probable IPAs in 37 hematologic patients (neutropenic in 36) were analyzed retrospectively. RESULTS The most frequent clinical signs associated with IPA were cough (92%), chest pain (76%), and hemoptysis (54%). Bronchoalveolar lavage (BAL) was positive in 22 of 32 cases. Aspergillus antigen test was positive in 83% of cases when tested on BAL fluid. Since October 1991, early thoracic computed tomographic (CT) scans were systematically performed in febrile neutropenic patients with pulmonary x-ray infiltrates. This approach allowed us to recognize suggestive CT halo signs in 92% of patients, compared with 13% before this date, and the mean time to IPA diagnosis was reduced dramatically from 7 to 1.9 days. Among 36 assessable patients, 10 failed to respond (amphotericin B [AmB] plus fluorocytosyne, n = 2; itraconazole + AmB, n = 8) and died of aspergillosis. Twenty-six patients were cured or improved by antifungal treatment (itraconazole with or without AmB, n = 22; voriconazole, n = 4). In 15 of 16 cases, surgical resection was combined successfully with medical treatment. Achievement of hematologic response, early diagnosis, unilateral pulmonary involvement, and highest level of fibrinogen value < 9 g/L were associated with better outcome. CONCLUSION In febrile neutropenic patients, systematic CT scan allows earlier diagnosis of IPA. Early antifungal treatment, combined with surgical resection if necessary, improves IPA prognosis dramatically in these patients.
Published: 1997

21. Central Nervous System Prophylaxis with High Dose Methotrexate. A Pharmacokinetic and Clinical Study: Results of Protocol FRALLE 89

Author: A. Baruchel, T. Leblanc, C. Schmitt, Yves Perel, Gérard Couillault, G. Schaison, I. Madelaine, and M. F. Auclerc
Subjects: Oncology, medicine.medical_specialty, business.industry, Central nervous system, Pharmacology, CNS Prophylaxis, High dose methotrexate, Clinical study, medicine.anatomical_structure, Pharmacokinetics, hemic and lymphatic diseases, Internal medicine, Medicine, Central nervous system leukemia, business, Childhood Acute Lymphoblastic Leukemia, Childhood all
Abstract: Cure of childhood acute lymphoblastic leukemia (ALL) can be achieved with increasing frequency (60–70%). Therapy to prevent CNS leukemia, so called CNS prophylaxis is an important element of the treatment of newly diagnosed children with ALL. Prolonged disease-free survival (DFS) in childhood ALL is dependent on therapy to prevent leukemic relapses in the CNS [5].
Published: 1994
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22. SFCE-P34 – Hématologie, immunologie – Anémie hémolytique auto-immune de l’enfant de moins de un an : étude retrospective de 41 enfants issus de de la cohorte française

Author: Y. Perel, Corinne Pondarré, Gérard Michel, F. Monpoux, Guy Leverger, Annie Robert, Brigitte Bader-Meunier, Christophe Piguet, Thierry Leblanc, C. Armari-Ala, Gérard Couillault, Virginie Gandemer, Jean-Louis Stephan, Pierre G. Lutz, and N. Aladjidi
Subjects: Pediatrics, Perinatology and Child Health
Abstract: Contexte Les anemies hemolytiques auto-immunes (AHAI) sont des affections hematologiques rares et de physiopathologie mal connue. Exceptionnelles chez les enfants de moins de un an, elles ont donc ete peu etudiees et leurs caracteristiques sont mal connues. Patients et Methodes Depuis Janvier 2003, une cohorte prospective de suivi des enfants atteints d’AHAI a ete mise en place en France par la Societe d’Hematologie et d’Immunologie Pediatrique (SHIP). Au 1er Septembre 2007, sur 267 enfants inclus, 41 etaient âge de moins de un an au diagnostic initial. Resultats L’âge median etait de 7 mois (0,8 a 11,7). 58 % etaient des garcons. Dans 10 % des cas, il existait une consanguinite, et dans 10 % des cas un antecedent familial hematologique (deficit en fas, AHAI, leucemie aigue, lupus). 20 % des enfants sont nes prematures. Au diagnostic initial, une infection virale etait documentee dans 19 % des cas. Dans 20 % des cas, la presentation clinique etait severe (choc, trouble de conscience, insuffisance renale aigue). Dans 24 % des cas une pathologie immunitaire etait associee (syndrome d’Evans (n = 5), anomalie de l’apoptose (n = 2), deficit en HLA de classe 2 (n = 1), hepatite a cellules geantes (n = 1), hypogammaglobulinemie (n = 1)). 87 % des enfants ont ete transfuses. Deux enfants n’ont pas recu de traitement specifique. Pour 21 des 39 enfants traites par corticoides (54 %), des traitements ulterieurs ont ete necessaires, avec un nombre median de lignes therapeutiques de 2 (0 a 7) : rituximab n = 14, ciclosporine n = 10, azathioprine n = 9, mycophenolate mofetil n = 4, splenectomie n = 3, allogreffe n = 2. Pour les 39 enfants traites, la duree mediane de traitement etait de 9,5 mois (0,9 a 64,6). Aux dernieres nouvelles, avec un recul median de 29,5 mois (0,5 a 115), 5 % des enfants sont decedes (un de maladie, un de toxicite), 31 % des enfants sont en remission complete sous traitement, et 61 % des enfants sont en remission complete sans traitement. Conclusion Les AHAI des enfants de moins de un an ont une presentation clinique initiale severe, et sont dependantes de traitements immunosuppresseurs lourds et prolonges. Plusieurs elements plaident en faveur d’une anomalie immunitaire constitutionnelle sous jacente.
Published: 2008
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23. SFCE-P19 – Cancérologie – Lymphome de Burkitt après transplantation d’organe en pédiatrie

Author: Pierre G. Lutz, C. Schmitt, Brigitte Nelken, Jacqueline Clavel, Gérard Couillault, Judith Landman-Parker, Yves Bertrand, Nizar Mahlaoui, Catherine Patte, S. Cohen Gogo, and Gérard Michel
Subjects: Pediatrics, Perinatology and Child Health
Abstract: Objectifs repertorier les lymphomes de Burkitt (LBu) survenant apres transplantation d’organe et analyser leurs particularites. Sujets/materiels et Methodes Apres contact avec les services pediatriques de greffe d’organe en France et avec le Registre National des Hemopathies de l’Enfant, 13 cas de LBu prouves histologiquement, de moins de 18 ans (a), ont ete repertories entre 1997 et 2007, puis analyses retrospectivement. Resultats 11 cas etaient evaluables : 9 garcons, 2 filles. Les transplantations etaient renales [2 patients (pts)] et hepatiques (9 pts). L’âge median a la greffe etait de 14 mois (m) (foie), de 8a 7 m (rein). La seroconversion EBV est survenue a une mediane de9 m apres la greffe, et de 20 m avant le LBu. Au diagnostic, les greffes renaux avaient une clairance moyenne de 50 ml/min/1,73 m2, 3 greffes hepatiques presentaient un rejet chronique. Le delai de survenue median du LBu etait de 2 a 9 m (foie), de 1a 11 m (rein). Les stades du lymphome etaient : stade III (6 pts), stade IV (1 pt), LAL L3 (4 pts). 10/11 pts avaient une charge virale sanguine EBV elevee au diagnostic. Le traitement immunosuppresseur a ete diminue dans tous les cas (pas de rejet observe), 3 pts ont recu du rituximab sans efficacite. 10/11 pts ont ete traites selon le protocole LMB 96 ou LMB 2001. 3 pts ont ete dialyses au diagnostic (1 greffe renal) mais un est decede de syndrome de lyse a J2 de traitement. Les complications infectieuses comprennent des septicemies bacteriennes documentees (5 pts), des atteintes fongiques (aspergillose pulmonaire, candidose hepatique : 2 pts), 1 abces pulmonaire (1 pt). 1 pt a presente une toxicite neurologique severe de l’aracytine a haute dose. Les 9 pts effectivement traites par le LMB sont en remission complete avec un recul median de 4 a. Le pt traite par un protocole moins intensif (prednisone, cyclophosphamide) est decede en rechute. Conclusions Le lymphome de Burkitt de l’enfant transplante peut etre gueri par un traitement intensif de type LMB mais les complications infectieuses peuvent etre plus frequentes et severes. Le lymphome et sa prise en charge semblent avoir peu d’influence sur le greffon (toxicite directe, rejet). Nous remercions vivement les medecins et chirurgiens des centres de greffe pour leur collaboration .
Published: 2008
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24. Childhood Standard-Risk B-Cell Precursor Acute Lymphoblastic Leukemia (SR-BCP-ALL): Toxicity and Efficacy Interim Results of the FRALLE 2000-A Protocol

Author: Brigitte Pautard, Gérard Michel, André Baruchel, Guy Leverger, Anne Auvrignon, Pascale Schneider, Catherine Paillard, Virginie Gandemer, Geneviève Marguerite, Claire Berger, Christiane Vermylen, Marie-Françoise Auclerc, Gérard Couillault, Christian Berthou, Francoise Mechinaud, Odile Lejars, Thierry Leblanc, Claudine Schmitt, Xavier Rialland, and Yves Perel
Subjects: education.field_of_study, medicine.medical_specialty, Asparaginase, Cyclophosphamide, business.industry, Incidence (epidemiology), Immunology, Population, Cell Biology, Hematology, medicine.disease, Biochemistry, Gastroenterology, Thrombosis, chemistry.chemical_compound, medicine.anatomical_structure, chemistry, Internal medicine, Toxicity, medicine, Pancreatitis, education, business, B cell, medicine.drug
Abstract: From December 2000 to May 2004, 433 children with SR-BCP-ALL (age: 1–9, WBC 1%). RESULTS: 1. Efficacy : no leukemic induction failure and a low incidence of patients with D21 M2/M3 marrow have been observed (5 % versus 13% in the FRALLE 93 for the same population). Only 4 out 319 evaluable pts (1.3%) have a high EOI MRD (≥ 1%) at the end of induction therapy. Five relapses have only been observed yet even if MFU is still short (31 months). 2.Toxicity: Seven cases of non leukemic deaths have been observed. Main grade III-IV toxicities were attributable to L-Aspa (CNS thrombosis: 5pts, pancreatitis: 8 cases, allergic reaction during DI °2: 35 pts). One case of secondary AML was observed (UPN98: no DNR) (25 months of CR) with translocation t(9;11) and MLL rearrangement. 3.Randomization: at this FU no difference between the DNR+ and DNR- arms is obvious neither in terms of toxicity nor efficacy. CONCLUSIONS: Most of the initial unusual toxicities mentioned where related to asparaginase even if potentially increased by the use of DEX during induction. Since most of the pancreatitis or CNS thrombosis episodes have been reported before July 2002, a potential hypothesis on the variation in the batches of the E.Coli asparaginase used in France can be made beside the classical ones (learning effect ....). Efficacy results, although preliminary, are very encouraging since at the same MFU 4 times more relapses (20 out of 410 pts) were observed in the FRALLE 93 for a comparable population.
Published: 2004
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25. Intermediate Dose Methotrexate in Low-Risk Acute Lymphoblastic Leukemia. Results of the FRALLE 93A Protocol

Author: André Baruchel, Hervé Chambost, Schneider Pascale, Jean-Paul Lamagnere, Christiane Vermylen, Mariane Debre, Thierry Leblanc, Guy Leverger, Yves Perel, Gérard Couillault, Virginie Gandemer, Marie-Françoise Auclerd, and Schmitt Claudine
Subjects: medicine.medical_specialty, Asparaginase, Vincristine, Cyclophosphamide, Cumulative dose, business.industry, Immunology, Cell Biology, Hematology, medicine.disease, Biochemistry, Gastroenterology, Surgery, chemistry.chemical_compound, chemistry, Internal medicine, Acute lymphocytic leukemia, medicine, Cytarabine, Vindesine, Methotrexate, business, medicine.drug
Abstract: The FRALLE 93A protocol aimed to identify a very low-risk ALL subgroup, to limit treatment morbidity and to investigate the value of intermediate dose methotrexate. Inclusion criteria were : B-cell precursor ALL, age 2-7 years, leukocyte count 181 children were included (13.5% of all the ALL included in the FRALLE 93 protocol) between June 1993 and Dec 1999. After a predisone prophase and induction phase (4-week daily prednisone, 4 weekly vincristine, thrice weekly 10,000 units of E coli asparaginase for 6 doses), during the 12-week antimetabolite consolidation therapy, patients were randomized to receive either low-dose methotrexate (25 mg/m2 p.o.) (lmtx) or 6 doses of intermediate dose methotrexate (IMTX) at 1500 mg/m2 by 24 hours infusion. Further therapy included a CCG type delayed intensification (dexamethasone, weekly vindesine, 3 doses of weekly doxorubicin and asparaginase, cytarabine) but without cyclophosphamide and a two years maintenance treatment. All pts received 16 triple intrathecal injections. The complete remission rate was 99.5%. The overall survival and event-free survival at 5 years were 93 % ± 4 % and 82 % ± 6 % (point date : 01.01.04). Only 1pt was considered prednisone-resistant (0.5%) D21 M1 marrow response is associated to a better 5 y EFS (86 + 5% vs 44 + 21% for M2/M3 pts (18 pts); p=0.001). Among 158 randomized patients, 9 testicular/meningeal relapses (6 isolated, 3 combined) occurred in the lmtx arm versus 1 in the IMTX arm (p=0.03) ; six BM relapses occurred in the lmtx group vs 9 in the IMTX group (p= NS). The 5-year DFS is not different 83% ± 8% and 85% ± 8% (p=0.47). Conclusion: a high cure rate could be achieved in low-risk ALL without cranial radiation, alkylating agents or epipodophyllotoxin and with a low cumulative dose of anthracyclins (75 mg/m2). Prednisone response is of no help in this subgroup of BCP-ALL. D21 marrow response allows to identify a very low-risk subgroup in addition to initial criteria. IMTX proved superior to lmtx in preventing testicular or meningeal relapse in low-risk ALL.
Published: 2004
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26. Increasing volume and changing characteristics of invasive pulmonary aspergillosis on sequential thoracic computed tomography scans in patients with neutropenia

Author: Gérard Couillault, Lionel Mannone, J. Lopez, F Piard, O. Vagner, Denis Caillot, A. Bernard, Olivier Casasnovas, J.-F. Couaillier, David W. Denning, and H. Guy
Subjects: Adult, Cancer Research, medicine.medical_specialty, Neutropenia, Adolescent, Aspergillosis, Statistics, Nonparametric, medicine, Humans, Child, Halo sign, Mycosis, Aged, Lung, Leukopenia, Lung Diseases, Fungal, business.industry, Respiratory disease, Middle Aged, medicine.disease, medicine.anatomical_structure, Aspergillus, Oncology, Child, Preschool, Hematologic Neoplasms, Radiology, medicine.symptom, Complication, business, Tomography, X-Ray Computed
Abstract: PURPOSE: In patients with neutropenia, thoracic computed tomography (CT) halo and air-crescent signs are recognized as major indicators of invasive pulmonary aspergillosis (IPA). Nevertheless, the exact timing of CT images is not well known. PATIENTS AND METHODS: Seventy-one thoracic CT scans were analyzed in 25 patients with neutropenia with surgically proven IPA. RESULTS: On the first day of IPA diagnosis with early CT scan (d0), a typical CT halo sign was observed in 24 of 25 patients. At that time, the median number of thoracic lesions was two (range, one to six), and pulmonary involvement was bilateral in 12 cases. The halo sign was present in 68%, 22%, and 19% of cases on d3, d7, and d14, respectively. Similarly, the air-crescent sign was seen in 8%, 28%, and 63% of cases on the same days. Otherwise, a nonspecific air-space consolidation aspect was seen in 31%, 50%, and 18% of cases on the same days. The analysis of calculated aspergillary volumes on CT showed that, despite antifungal treatment, the median volume of lesions increased four-fold from d0 to d7, whereas it remained stable from d7 to d14. Overall, 21 patients (84%) were cured by the medical-surgical approach. CONCLUSION: In patients with neutropenia, CT halo sign is a highly effective modality for IPA diagnosis. The duration of the halo sign is short, and it demonstrates the value of early CT. The increase of the aspergillosis size on CT in the first days after IPA diagnosis is not correlated with a pejorative immediate outcome when using a combined medical-surgical approach.

27. Daunorubicin or Not During the Induction Treatment of Childhood Standard-Risk B-Cell Precursor Acute Lymphoblastic Leukemia (SR-BCP-ALL): The Randomized Fralle 2000-A Protocol

Author: Virginie Gandemer, Nathalie Grardel, Yves Perel, Anne Auvrignon, Benoit Brethon, Sylvie Chevret, Caroline Thomas, Christian Berthou, Kheira Beldjord, Christophe Piguet, Francoise Mechinaud, Elizabeth Macintyre, Catherine Paillard, Gérard Michel, Claire Berger, Brigitte Pautard, Christiane Vermylen, André Baruchel, Marie-Françoise Auclerc, Pascale Schneider, Isabelle Pellier, Jean-Michel Cayuela, Geneviève Margueritte, Guy Leverger, Arnaud Petit, Odile Lejars, Thierry Leblanc, Gérard Couillault, Claudine Schmitt, and Jean Soulier
Subjects: education.field_of_study, Chemotherapy, medicine.medical_specialty, Univariate analysis, Pediatrics, Asparaginase, Randomization, Daunorubicin, business.industry, medicine.medical_treatment, Immunology, Population, Cell Biology, Hematology, Hematopoietic stem cell transplantation, Biochemistry, Gastroenterology, chemistry.chemical_compound, chemistry, Prednisone, Internal medicine, medicine, education, business, medicine.drug
Abstract: Abstract 135 From December 2000 to June 2010, 1201 children with SR-BCP-ALL (age: 1–9 years, WBC Results: 1. Overall efficacy: No leukemic induction failure was observed; 4 induction deaths (0.3%) occurred leading to a 99.7% CR rate. Only 30 out 1097 evaluable pts (2.7%) had a decisional EOI MRD ≥ 1%. Eighty-two relapses have been observed (BM: 47, CNS+: 24, BM+CNS: 9, testis: 2). For the whole population 5-year EFS is 91.5% (95%CI: 89.8–93.3), 5-year OS is 97.4% (95%CI: 96.4–98.4). 5-yr EFS and OS for the A1 and A3 groups are 93.1% vs. 61.2% (p 2. Overall toxicity: 5 non leukemic deaths have been observed after CR (0.4%). Other main toxicities were attributable to ASPA (CNS thrombosis: 1.5%, grade 3–4 pancreatitis: 0.8%, allergic reaction-all grades- during DI °2: 70%). Six cases of secondary neoplasms were observed (AML: 5 pts, astrocytoma: 1 pt). 3. Randomization: 5y EFS and OS from randomization was 92.9% and 97.2% for D21 M1 pts DNR(+) versus 93.3% and 98.2% for D21 M1 DNR(-) pts (p= 0.89 and p= 0.85, respectively). Interestingly EOI MRD levels in the two arms were not different at the sensitivity cut-off of 10−2 (p=0.93) or 10−3(p= 0.74). 4. Prognostic factors: For the whole population, age < 6 years, WBC < 20.000/mm3, ETV6-RUNX1 positivity, as well as D21 marrow M1 and EOI MRD levels of 10−2 and 10−3 were all associated to EFS in univariate analysis. In multivariate analysis remain only WBC < 20.000/mm3 (p=.001), ETV6-RUNX1 positivity (p=.02), EOI MRD levels ≤ 10−3 (p=1.4×10−5). Considering only randomized pts (D21M1 pts) age Conclusions: Very good results were obtained with this VCR-DEX-ASPA oriented protocol. SR-BCP ALL pts which represents around 55 % of all children with ALL can be cured with a modest dose of anthracyclin (75 mg/m2). A further decrease in therapy based on the identified prognostic factors could be proposed. Intensification of the chemotherapy for the rare SR pts with a very high MRD has salvaged half of these pts but the addition of new drugs and/or HSCT is now to be evaluated. Disclosures: No relevant conflicts of interest to declare.

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27 results on '"Gérard Couillault"'

1. An appraisal of the frequency and severity of noninfectious manifestations in primary immunodeficiencies: A study of a national retrospective cohort of 1375 patients over 10 years

2. Genetic diagnosis of primary immunodeficiencies: A survey of the French national registry

3. Growth deceleration in children treated with imatinib for chronic myeloid leukaemia

4. Les leucémies aiguës lymphoblastiques congénitales : expérience française entre 1990 et 2010

5. Development of primary early-onset colorectal cancers due to biallelic mutations of the FANCD1/BRCA2 gene

6. Prospective survey of indoor fungal contamination in hospital during a period of building construction

7. Age and high-dose methotrexate are associated to clinical acute encephalopathy in FRALLE 93 trial for acute lymphoblastic leukemia in children

8. Lymphocyte-Predominant Hodgkin’s Lymphoma in Children: Therapeutic Abstention After Initial Lymph Node Resection—A Study of the French Society of Pediatric Oncology

9. Autoimmune and inflammatory manifestations occur frequently in patients with primary immunodeficiencies

10. Human RTEL1 deficiency causes Hoyeraal-Hreidarsson syndrome with short telomeres and genome instability

11. Efficacy of vinblastine in central nervous system Langerhans cell histiocytosis: a nationwide retrospective study

12. Imatinib is effective in children with previously untreated chronic myelogenous leukemia in early chronic phase: results of the French national phase IV trial

13. Profiles and seasonal distribution of airborne fungi in indoor and outdoor environments at a French hospital

14. Spontaneous macrophage colony-forming units in juvenile myelomonocytic leukaemia

15. Hypereosinophilia in acute B-lineage lymphoblastic leukaemia

16. 'Agranular CD4+ CD56+ hematodermic neoplasm' (blastic NK-cell lymphoma) originates from a population of CD56+ precursor cells related to plasmacytoid monocytes

17. SFCE CO-09 - Syndrome CMMR-D : description clinique dans une série française

18. Acute Myeloid Leukemia (AML) in Children: Results of the French Trials

19. Decreased Activity of Clotting Factor V During ALL Consolidation Treatment with Mercaptopurine in a Child

20. Improved management of invasive pulmonary aspergillosis in neutropenic patients using early thoracic computed tomographic scan and surgery

21. Central Nervous System Prophylaxis with High Dose Methotrexate. A Pharmacokinetic and Clinical Study: Results of Protocol FRALLE 89

22. SFCE-P34 – Hématologie, immunologie – Anémie hémolytique auto-immune de l’enfant de moins de un an : étude retrospective de 41 enfants issus de de la cohorte française

23. SFCE-P19 – Cancérologie – Lymphome de Burkitt après transplantation d’organe en pédiatrie

24. Childhood Standard-Risk B-Cell Precursor Acute Lymphoblastic Leukemia (SR-BCP-ALL): Toxicity and Efficacy Interim Results of the FRALLE 2000-A Protocol

25. Intermediate Dose Methotrexate in Low-Risk Acute Lymphoblastic Leukemia. Results of the FRALLE 93A Protocol

26. Increasing volume and changing characteristics of invasive pulmonary aspergillosis on sequential thoracic computed tomography scans in patients with neutropenia

27. Daunorubicin or Not During the Induction Treatment of Childhood Standard-Risk B-Cell Precursor Acute Lymphoblastic Leukemia (SR-BCP-ALL): The Randomized Fralle 2000-A Protocol

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27 results on '"Gérard Couillault"'

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