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1. Real-world use of defibrotide for veno-occlusive disease/sinusoidal obstruction syndrome: the DEFIFrance Registry Study

Author: Mohamad Mohty, Didier Blaise, Régis Peffault de Latour, Myriam Labopin, Jean Henri Bourhis, Benedicte Bruno, Patrice Ceballos, Marie Detrait, Virginie Gandemer, Anne Huynh, Faezeh Izadifar-Legrand, Charlotte Jubert, Hélène Labussière-Wallet, Delphine Lebon, Sébastien Maury, Catherine Paillard, Cécile Pochon, Cecile Renard, Fanny Rialland, Pascale Schneider, Anne Sirvent, Kobby Asubonteng, Gwennaëlle Guindeuil, Ibrahim Yakoub-Agha, Jean-Hugues Dalle, CHU Saint-Antoine [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Centre de Recherche Saint-Antoine (CRSA), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU), Centre de Recherche en Cancérologie de Marseille (CRCM), Aix Marseille Université (AMU)-Institut Paoli-Calmettes, Fédération nationale des Centres de lutte contre le Cancer (FNCLCC)-Fédération nationale des Centres de lutte contre le Cancer (FNCLCC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Institut Paoli-Calmettes, Fédération nationale des Centres de lutte contre le Cancer (FNCLCC), Département d'hématologie [Gustave Roussy], Institut Gustave Roussy (IGR), Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Hôpital Lapeyronie [Montpellier] (CHU), Service d'Hématologie [CHRU Nancy], Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Institut de Génétique et Développement de Rennes (IGDR), Université de Rennes (UR)-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), Groupe hospitalier Pellegrin, Centre Hospitalier Lyon Sud [CHU - HCL] (CHLS), Hospices Civils de Lyon (HCL), CHU Amiens-Picardie, HEMATIM - Hématopoïèse et immunologie - UR UPJV 4666 (HEMATIM), Université de Picardie Jules Verne (UPJV)-CHU Amiens-Picardie-Institut National de la Santé et de la Recherche Médicale (INSERM), Université Paris-Est Créteil Val-de-Marne - Faculté de médecine (UPEC Médecine), Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), Hôpital Henri Mondor, 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, Service d'Oncologie Pédiatrique [CHRU Nancy], Service d’Hématologie [Centre Hospitalier Lyon Sud - HCL], Hospices Civils de Lyon (HCL)-Hospices Civils de Lyon (HCL), Centre hospitalier universitaire de Nantes (CHU Nantes), Service de pédiatrie médicale et médecine de l'adolescent [Rouen], CHU Rouen, Normandie Université (NU)-Normandie Université (NU)-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU), Physiopathologie, Autoimmunité, maladies Neuromusculaires et THErapies Régénératrices (PANTHER), Université de Rouen Normandie (UNIROUEN), Normandie Université (NU)-Normandie Université (NU)-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Montpellier, Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Jazz Pharmaceuticals, Inc, Jazz Pharmaceuticals [Lyon], Institute for Translational Research in Inflammation - U 1286 (INFINITE (Ex-Liric)), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Hôpital Robert Debré Paris, Hôpital Robert Debré, and Jazz Pharmaceuticals
Subjects: Transplantation, Haematopoietic stem cells, Hematology, Drug therapy, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology
Abstract: Veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS) is a potentially life-threatening complication of haematopoietic cell transplantation (HCT) conditioning. The DEFIFrance post-marketing registry study evaluated effectiveness and safety in patients who received defibrotide. It collected retrospective/prospective patient data from 53 French HCT centres from July 2014 to March 2020. Primary endpoints were survival and complete response (CR; total serum bilirubin
Published: 2022
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2. Cerebellar swelling after surgery for medulloblastoma with leptomeningeal dissemination in children. A case based-update

Author: P. Chastagner, Hugo-Andres Coca, O. Klein, François Proust, Natacha Entz-Werle, A. Joud, J. Todeschi, I. Stella, Service de Neurochirurgie Pédiatrique [CHRU Nancy], Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Service de Neurochirurgie [HUS, Strasbourg], Les Hôpitaux Universitaires de Strasbourg (HUS), Laboratoire de Bioimagerie et Pathologies (LBP), Université de Strasbourg (UNISTRA)-Centre National de la Recherche Scientifique (CNRS), CHU Strasbourg, Service d'Oncologie Pédiatrique [CHRU Nancy], and univOAK, Archive ouverte
Subjects: Upward herniation, Male, medicine.medical_specialty, [SDV]Life Sciences [q-bio], Fourth ventricle, Large Cell Medulloblastoma, Anaplastic Medulloblastoma, 03 medical and health sciences, Fatal Outcome, Postoperative Complications, 0302 clinical medicine, Cerebellum, Humans, Medicine, Cerebellar Neoplasms, Child, Pathological, Medulloblastoma, Fourth Ventricle, business.industry, Leptomeningeal diffusion, Cerebellar swelling, medicine.disease, Gross Total Resection, Surgery, Hydrocephalus, [SDV] Life Sciences [q-bio], Child, Preschool, 030220 oncology & carcinogenesis, Female, Neurology (clinical), Intracranial Hypertension, business, Complication, 030217 neurology & neurosurgery
Abstract: Object Despite the improvement in the overall management of medulloblastomas in recent years, certain phenomena and in particular postoperative cerebellar swelling remain an enigma. This rare complication, little described in the literature, is nonetheless life threatening for the patients. Case reports We report our experience about two children who developed severe cerebellar swelling with hydrocephalus and upward herniation soon after a gross total resection of a fourth ventricle medulloblastoma by a telo-velar approach. Despite rapid management of ventricular dilation and optimal medical intensive treatment of intracranial hypertension, both children died quickly after the surgery. Pathological examination analyses were in favour of anaplastic/large cell medulloblastoma. Discussion Diffuse cerebellar swelling with upward herniation may occur postoperatively in young children with anaplastic/large cell medulloblastoma with leptomeningeal spread. In the literature, only 4 cases have been so far described with delayed onset of symptoms. Two children survived with an aggressive management (decompressive surgery and early radio-chemotherapy). Conclusion Cerebellar swelling is an unrecognised and sudden complication of posterior fossa surgery for metastatic anaplastic medulloblastoma with leptomeningeal dissemination in young children. An initial less invasive surgical approach could be considered in such cases, in order to prevent this complication with potentially tragic issue, and which cannot be managed with a CSF shunt alone.
Published: 2021
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3. Complications des cellules CAR-T autres que les infections, CRS et ICANS : recommandations de la Société francophone de greffe de moelle et de thérapie cellulaire (SFGM-TC)

Author: Cécile, Pochon, Corinne, Courbon, Jacques-Olivier, Bay, Anne-Sophie, Moreau, Franciane, Paul, Muriel, Picard, Arthur, Sterin, Jean-Jacques, Tudesq, Céline, Vicente, Mathilde, Yakoub-Agha, Ibrahim, Yakoub-Agha, Service d'Oncologie Pédiatrique [CHRU Nancy], Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Ingénierie Moléculaire et Physiopathologie Articulaire (IMoPA), Université de Lorraine (UL)-Centre National de la Recherche Scientifique (CNRS), Institut de Cancérologie Lucien Neuwirth, Centre Hospitalier Universitaire de Saint-Etienne [CHU Saint-Etienne] (CHU ST-E), CHU Clermont-Ferrand, Hôpital Roger Salengro [Lille], Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Université de Montpellier (UM), 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 de la Timone [CHU - APHM] (TIMONE), CHU Amiens-Picardie, Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Institute for Translational Research in Inflammation - U 1286 (INFINITE (Ex-Liric)), and Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille)
Subjects: Neutropenia, Receptors, Chimeric Antigen, Heart Diseases, CAR-T cells, Incidence, T-Lymphocytes, GVHD, Graft vs Host Disease, [SDV.MHEP.HEM]Life Sciences [q-bio]/Human health and pathology/Hematology, [SDV.IMM.IMM]Life Sciences [q-bio]/Immunology/Immunotherapy, Immunotherapy, Adoptive, Risk Factors, Humans, tumor lysis syndrome, cardio-vascular complications, delayed cytopenias
Abstract: International audience; Texte de l'atelier Rappel du titre de l'atelier : Complications des cellules CAR T autres que les infections, CRS et ICANS: recommandations de la Société Francophone de greffe de moelle et de thérapie cellulaire (SFGM-TC) Résumé en Français : Les cellules CART sont un traitement innovant, qui s'adresse à un nombre croissant de patients, en particulier depuis l'élargissement des indications au lymphome du manteau et au myélome multiple. Certaines complications, jugées rares, ont cependant été décrites de façon itérative pour des petites séries de patients, du fait de l'augmentation du nombre de patients traités dans le monde depuis les premiers essais cliniques en 2011. Parmi elles, le syndrome de lyse tumorale, les complications cardiaques, les cytopénies différées ou prolongées, la maladie du greffon contre l'hôte aiguë ou chronique, et font actuellement l'objet d'une reconnaissance en tant que complications potentielles et spécifiques du traitement par cellules CART. Nous détaillons ici l'épidémiologie, la physiopathologie connue ou suspectée, les facteurs de risque de ces complications, et proposons des recommandations pratiques de leur prévention et de leur prise en charge. Ces recommandations s'adressent aux cellules CART commerciales. Mots clés : CART cells, syndrome de lyse tumorale, complications cardio-vasculaires, cytopénies retardées, GVHD, Résumé en anglais : CAR T cells are an innovative treatment for an increasing number of patients, particularly since the extension of their indication to mantle lymphoma and multiple myeloma. Several complications of CAR T-cell therapy, that were first described as exceptional, have now been reported in series of patients, since its first clinical use in 2011. Among them, cardiac complications, delayed cytopenias, acute and chronic Graft versus Host Disease, and tumoral lysis syndrome are recognized as specific potent complications following CAR T-cells infusion. During the twelfth edition of practice harmonization workshops of the Francophone society of bone marrow transplantation and cellular therapy (SFGM-TC), a working group focused its work on the management of these complications with focuses the epidemiology, the physiopathology and the risk factors of these 4 side effects. Our recommendations apply to commercial CART cells, in order to guide strategies for the management of complications associated with this new therapeutic approach.
Published: 2021
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4. Improved outcome in children compared to adolescents and young adults after allogeneic hematopoietic stem cell transplant for acute myeloid leukemia: a retrospective study from the Francophone Society of Bone Marrow Transplantation and Cell Therapy (SFGM-TC)

Author: Marie Angoso, Yves Chalandon, Anne Huynh, Justyna Kanold, Thomas Remen, Cécile Pochon, Anne Sirvent, Eolia Brissot, Faezeh Izzadifar-Legrand, Yves Beguin, Edouard Forcade, Bénédicte Neven, Stéphanie Nguyen, Eliane Albuisson, Marie-Thérèse Rubio, Patrice Chevallier, Marie Balza, Mauricette Michallet, Anne-Lise Ménard, Fanny Rialland, Marie Y Detrait, Nicole Raus, Jean-Hugues Dalle, Cecile Renard, Fanny Gonzales, Catherine Paillard, Jacques-Olivier Bay, Claude Eric Bulabois, Gérard Michel, Pascale Schneider, Ibrahim Yakoub-Agha, Nathalie Dhedin, Jérôme Cornillon, Ali Bazarbachi, Service d'Oncologie Pédiatrique [CHRU Nancy], Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Ingénierie Moléculaire et Physiopathologie Articulaire (IMoPA), Université de Lorraine (UL)-Centre National de la Recherche Scientifique (CNRS), Service d'Hématologie [CHRU Nancy], Service d'Hématologie Biologique [Hôpital Robert Debré, Paris], AP-HP Hôpital universitaire Robert-Debré [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Hôpital de la Timone [CHU - APHM] (TIMONE), Hopital Saint-Louis [AP-HP] (AP-HP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Hôpitaux Universitaires de Genève (HUG), CHU Saint-Antoine [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Centre de Recherche Saint-Antoine (CRSA), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU), CHU Bordeaux [Bordeaux], Hôpital Arnaud de Villeneuve [CHRU Montpellier], Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Institut Paoli-Calmettes, Fédération nationale des Centres de lutte contre le Cancer (FNCLCC), Centre Léon Bérard [Lyon], Hospices Civils de Lyon (HCL), Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Institute for Translational Research in Inflammation - U 1286 (INFINITE (Ex-Liric)), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), CHU Clermont-Ferrand, CHU Estaing [Clermont-Ferrand], Institut de Cancérologie Lucien Neuwirth, Centre Hospitalier Universitaire de Saint-Etienne [CHU Saint-Etienne] (CHU ST-E), Centre Hospitalier Universitaire [Grenoble] (CHU), Centre d'Immunologie et des Maladies Infectieuses (CIMI), Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU)-Centre National de la Recherche Scientifique (CNRS), Centre hospitalier universitaire de Nantes (CHU Nantes), American University of Beirut [Beyrouth] (AUB), Centre Hospitalier Universitaire de Liège (CHU-Liège), 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), Centre de Lutte Contre le Cancer Henri Becquerel Normandie Rouen (CLCC Henri Becquerel), Hôpital Charles Nicolle [Rouen], CHU Rouen, Normandie Université (NU)-Normandie Université (NU), CHU Necker - Enfants Malades [AP-HP], Hôpital de Hautepierre [Strasbourg], Centre Hospitalier Lyon Sud [CHU - HCL] (CHLS), Délégation à la Recherche Clinique et à l'Innovation [CHRU Nancy] (DRCI), Service d'hématologie clinique et de thérapie cellulaire [CHU Saint-Antoine], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Saint-Antoine [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU), CHU Saint-Etienne, Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Institut National de la Santé et de la Recherche Médicale (INSERM), Gestionnaire, Hal Sorbonne Université, Centre de Recherche Saint-Antoine (CR Saint-Antoine), Sorbonne Université (SU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Saint-Antoine [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Centre d'Immunologie et de Maladies Infectieuses (CIMI), and Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-CHU Toulouse [Toulouse]-Institut National de la Santé et de la Recherche Médicale (INSERM)
Subjects: Cancer Research, medicine.medical_specialty, Transplantation Conditioning, Adolescent, Acute myeloblastic leukemia, Graft vs Host Disease, [SDV.CAN]Life Sciences [q-bio]/Cancer, Young Adult, 03 medical and health sciences, 0302 clinical medicine, [SDV.CAN] Life Sciences [q-bio]/Cancer, Chronic GVHD, Internal medicine, medicine, Humans, Cumulative incidence, Risk factor, Young adult, Child, Children, Bone Marrow Transplantation, Retrospective Studies, Outcome, ddc:616, Hematology, Acute GVHD, business.industry, Hematopoietic Stem Cell Transplantation, Retrospective cohort study, General Medicine, medicine.disease, Adolescent and post-adolescent patients, 3. Good health, Transplantation, Leukemia, Myeloid, Acute, medicine.anatomical_structure, Oncology, 030220 oncology & carcinogenesis, Allogeneic hematopoietic stem cell transplantation, Bone marrow, business, 030215 immunology, Young adults
Abstract: Background There are currently few data on the outcome of acute myeloid leukemia (AML) in adolescents after allogeneic HSCT. The aim of this study is to describe the outcome and its specific risk factors for children, adolescents and young adults after a first allogeneic HSCT for AML. Methods In this retrospective study, we compared the outcome of AML patients receiving a first allogeneic HSCT between 2005 and 2017 according to their age at transplantation’s time: children (n = 564), adolescent and post-adolescent (APA) patients (15–25 years, n = 647) and young adults (26–40 years; n = 1434). Results With a median follow-up of 4.37 years (min–max 0.18–14.73 years), the probability of 2-year overall survival (OS) was 71.4% in children, 61.1% in APA patients and 62.9% in young adults (p = 0.0009 for intergroup difference). Both relapse and non-relapse mortality (NRM) Cumulative Incidence (CI) estimated at 2 years were different between the age groups (30.8% for children, 35.2% for APA patients and 29.4% for young adults—p = 0.0254, and 7.0% for children, 10.6% for APA patients and 14.2% for young adults, p p Conclusion Age is an independent risk factor for NRM and extensive chronic GVHD. This study suggests that APA patients with AML could be beneficially treated with a chemotherapy-based MAC regimen and bone marrow as a stem cells source.
Published: 2021
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5. Testosterone deficiency in men surviving childhood acute leukemia after treatment with hematopoietic stem cell transplantation or testicular radiation: an L.E.A. study

Author: André Baruchel, Nicolas Sirvent, Geneviève Plat, Jean-Hugues Dalle, Cécile Pochon, Dominique Plantaz, Justyna Kanold, Marie-Dominique Tabone, Pascal Auquier, Gérard Michel, Stéphane Ducassou, Guy Leverger, Julie Berbis, Zeinab Hamidou, Isabelle Pellier, Sophie Ansoborlo, Marilyne Poirée, Sandrine Thouvenin, Paul Saultier, Romain Lopez, Catherine Paillard, Virginie Gandemer, Yves Bertrand, Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Hospices Civils de Lyon (HCL), CHU Bordeaux [Bordeaux], Pédiatrie et oncologie pédiatrique [Hôpital de la Timone - APHM], Aix Marseille Université (AMU)-Assistance Publique - Hôpitaux de Marseille (APHM)- Hôpital de la Timone [CHU - APHM] (TIMONE), Centre d'études et de recherche sur les services de santé et la qualité de vie (CEReSS), Aix Marseille Université (AMU), Service d'Oncologie Pédiatrique [CHRU Nancy], Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Centre Hospitalier Universitaire de Nice (CHU Nice), CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), CHU Estaing [Clermont-Ferrand], CHU Clermont-Ferrand, Service d'Hématologie Biologique [Hôpital Robert Debré, Paris], AP-HP Hôpital universitaire Robert-Debré [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), 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 ), Les Hôpitaux Universitaires de Strasbourg (HUS), Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Hôpital Couple Enfant de Grenoble, Centre Hospitalier Universitaire de Saint-Etienne [CHU Saint-Etienne] (CHU ST-E), Centre Hospitalier Universitaire d'Angers (CHU Angers), PRES Université Nantes Angers Le Mans (UNAM), Institut National Du Cancer, Association Laurette Fugain, Agence Nationale de la Recherche, Ligue Contre le Cancer, and Canceropôle PACA
Subjects: Male, medicine.medical_specialty, Myeloid, Transplantation Conditioning, medicine.medical_treatment, Graft vs Host Disease, Hematopoietic stem cell transplantation, Gastroenterology, [SDV.BDLR.RS]Life Sciences [q-bio]/Reproductive Biology/Sexual reproduction, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, Medicine, Humans, Testosterone, Child, Busulfan, ComputingMilieux_MISCELLANEOUS, Transplantation, Acute leukemia, Leydig cell, business.industry, Hematopoietic Stem Cell Transplantation, [SDV.MHEP.HEM]Life Sciences [q-bio]/Human health and pathology/Hematology, Hematology, Total body irradiation, [SDV.MHEP.EM]Life Sciences [q-bio]/Human health and pathology/Endocrinology and metabolism, medicine.disease, Leukemia, Leukemia, Myeloid, Acute, medicine.anatomical_structure, 030220 oncology & carcinogenesis, Metabolic syndrome, business, Whole-Body Irradiation, 030215 immunology
Abstract: We included 255 patients from the L.E.A. French long-term follow-up cohort. All had received hematopoietic stem cell transplantation (HSCT) and/or testicular radiation for childhood acute leukemia and were older than 18 years at last L.E.A. evaluation. Total testosterone deficiency was defined as a12 nmol/l level or by substitutive therapy, partial deficiency as normal testosterone with elevated luteinizing hormone (10 UI/l). After myeloablative total body irradiation (n = 178), 55.6% had total deficiency, 15.7% partial deficiency, and 28.7% were normal. A 4-6 Gy testicular boost and a younger age at HSCT increased significantly the risk. After a Busulfan-containing myeloablative conditioning regimen (n = 53), 28.3% had total deficiency, 15.1% partial deficiency, 56.6% were normal (62.5% vs. 0% in patients without or with additional testicular radiation). A 24-Gy testicular radiation without HSCT induced total or partial deficiency in 71.4% and 28.6%, respectively (n = 21). Total testosterone deficiency increased the risk of metabolic syndrome: 25% vs. 12.1% in men with partial testosterone deficiency and 8.8% when Leydig cell function was normal (p = 0.031).
Published: 2020
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6. Rapid fully-automated assay for routine molecular diagnosis of BRAF mutations for personalized therapy of low grade gliomas

Author: François Doz, Marie Rouyer, Agnès Leroux, Guillaume Gauchotte, Jean-Louis Merlin, Pascal Chastagner, Pascale Varlet, Marie-Sophie Merlin, Pauline Gilson, Centre de Recherche en Automatique de Nancy (CRAN), Centre National de la Recherche Scientifique (CNRS)-Université de Lorraine (UL), Institut de Cancérologie de Lorraine - Alexis Vautrin [Nancy] (UNICANCER/ICL), UNICANCER, Service d'Oncologie Pédiatrique [CHRU Nancy], Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Institut Curie [Paris], Centre Hospitalier Sainte Anne [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Nutrition-Génétique et Exposition aux Risques Environnementaux (NGERE), and Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lorraine (UL)
Subjects: Oncology, medicine.medical_specialty, business.industry, Hematology, Automated real-time PCR, 3. Good health, BRAF, [SPI.AUTO]Engineering Sciences [physics]/Automatic, 03 medical and health sciences, 0302 clinical medicine, Fully automated, 030220 oncology & carcinogenesis, Internal medicine, Pediatrics, Perinatology and Child Health, molecular diagnosis, medicine, Low-Grade Glioma, Personalized therapy, business, low grade glioma, neoplasms, 030215 immunology
Abstract: International audience; Background: BRAF mutation analysis is important to personalize the management with low-grade gliomas (LGG) in children and adults, with therapeutic and prognostic impacts. In recurrent tumors, targeted therapies such as BRAF inhibitors had been reported to induce disease stabilization and significant radiographic responses. This highlights the potential interest of BRAF mutation to stratify patients for targeted therapy. Standard operating procedures (SOP) for BRAF V600E mutation detection can be time-consuming and consequently delay treatment choice in patients with acute deterioration. Here, we evaluated IdyllaTM fully automated PCR (FA-PCR) assay for the rapid determination of BRAF mutational status in children and adult LGG.Methods: Formalin-fixed and paraffin-embedded (FFPE) samples from three histological LGG subtypes (ganglioglioma, pleomorphic xantoastrocytoma, and dysembryoplastic neuroepithelial tumor) with previous SOP-characterized BRAF mutational status were re-analyzed using the FA-PCR. Overall concordance with the mutational status determined using SOP, as well as sensitivity and specificity of FA-PCR technique were assessed.Results: All 14 samples gave interpretable results with FA-PCR. Overall concordance of BRAF mutational status between FA-PCR and SOP was 100%. Sensitivity and specificity were 100%.Conclusion: This study confirms the reliability of FA-PCR for BRAF mutations analysis in children and adult LGG. Considering the short time to results enabled by FA-PCR, providing results in less than 90 minutes, this technique represents an interesting option for the molecular diagnosis of LGG and personalization of treatment.
Published: 2020
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7. SFCE METRO‐01 four‐drug metronomic regimen phase II trial for pediatric extracranial tumor

Author: Heng-Maillard, Marie-Amélie, Verschuur, Arnauld, Aschero, Audrey, Dabadie, Alexia, Jouve, Elisabeth, Chastagner, Pascal, Leblond, Pierre, Aerts, Isabelle, de Luca, Bénédicte, André, Nicolas, Heng‐maillard, Marie‐amélie, Luca, Bénédicte, Pédiatrie et oncologie pédiatrique [Hôpital de la Timone - APHM], Aix Marseille Université (AMU)-Assistance Publique - Hôpitaux de Marseille (APHM)- Hôpital de la Timone [CHU - APHM] (TIMONE), Centre d'Investigation Clinique [Hôpital de la Conception - APHM] (CIC), Aix Marseille Université (AMU)-Assistance Publique - Hôpitaux de Marseille (APHM)-Institut Paoli-Calmettes, Fédération nationale des Centres de lutte contre le Cancer (FNCLCC)-Fédération nationale des Centres de lutte contre le Cancer (FNCLCC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Hôpital de la Conception [CHU - APHM] (LA CONCEPTION), Service d'Oncologie Pédiatrique [CHRU Nancy], Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Centre Régional de Lutte contre le Cancer Oscar Lambret [Lille] (UNICANCER/Lille), Université de Lille-UNICANCER, Institut Curie [Paris], Aix-Marseille Université - Faculté de pharmacie (AMU PHARM), Aix Marseille Université (AMU), Centre de Recherche en Cancérologie de Marseille (CRCM), Aix Marseille Université (AMU)-Institut Paoli-Calmettes, Fédération nationale des Centres de lutte contre le Cancer (FNCLCC)-Fédération nationale des Centres de lutte contre le Cancer (FNCLCC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), and Université Lille Nord de France (COMUE)-UNICANCER
Subjects: Male, [SDV]Life Sciences [q-bio], Gastroenterology, Hemangioendothelioma, extracranial solid tumor, angiogenesis, 0302 clinical medicine, Neoplasms, MESH: Child, metronomic chemotherapy, myoepithelial carcinoma, MESH: Neoplasms, Child, Hematology, pediatric oncology, 3. Good health, Vinblastine, Survival Rate, epithelioid hemangioendothelioma, MESH: Administration, Metronomic, Oncology, 030220 oncology & carcinogenesis, Child, Preschool, Female, Sarcoma, medicine.drug, Adult, medicine.medical_specialty, Adolescent, MESH: Survival Rate, [SDV.BC]Life Sciences [q-bio]/Cellular Biology, Neutropenia, Models, Biological, Disease-Free Survival, 03 medical and health sciences, Internal medicine, medicine, Humans, MESH: Adolescent, MESH: Humans, business.industry, MESH: Child, Preschool, MESH: Celecoxib, MESH: Models, Biological, MESH: Adult, medicine.disease, Metronomic Chemotherapy, MESH: Male, Regimen, Celecoxib, Pediatrics, Perinatology and Child Health, Administration, Metronomic, MESH: Disease-Free Survival, Methotrexate, business, MESH: Female, Progressive disease, 030215 immunology
Abstract: Objective To investigate the antitumor activity of a four-drug metronomic chemotherapy (MC) regimen in relapsed/progressing pediatric extracranial solid tumors (EST). The primary objective was clinical benefit (complete response /partial response/stable disease [SD]) after two cycles of therapy (four months). Methods Patients aged ≥4 to 25 years with progressing EST and adequate organ function were eligible. Treatment consisted of an eight-week cycle of oral celecoxib b.i.d., weekly vinblastine, and oral cyclophosphamide for three weeks alternating with oral methotrexate for three weeks, with a two-week rest. The Kepner-Chang two-stage model was used with 10 patients in the first stage. If primary objective was reached in two or more patients, eight additional patients were included according to four groups: neuroblastoma (NBL), soft-tissue sarcoma (STS), bone sarcoma (BS), and miscellaneous (Misc.). Results Forty-four patients were evaluable. The NBL cohort could be expanded to 18 patients: 4 of 18 patients stabilized with MC treatment for 6 (n = 1) and 12 (n = 3) months. In STS, two of seven patients (metastatic hemangioendothelioma and angiosarcoma) had SD for > 2 cycles. One of nine Misc. (metastatic myoepithelial carcinoma) had SD for one year. All patients with BS had progressive disease. One-year progression-free survival of the whole cohort was 6.8% and one-year overall survival was 55.3%. Grade 3 toxicity occurred in 18 patients and grade 4 in 15 patients. The most frequent toxicity was hematologic, predominantly neutropenia. Conclusions This MC has no activity in BS and limited though interesting activity in NBL with some patients being stable for > 1 year. It is not possible to conclude activity in STS and Misc.
Published: 2019
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8. Adolescence and Socioeconomic Factors: Key Factors in the Long-Term Impact of Leukemia on Scholastic Performance—A LEA Study

Author: Sandrine Thouvenin, Nicolas Sirvent, André Baruchel, Jacinthe Bonneau, Catherine Paillard, Camille Vercasson, Pascal Auquier, Marie Dominique Tabone, Yves Bertrand, Virginie Gandemer, Maryline Poirée, Jean-Hugues Dalle, Paul Saultier, Audrey Contet, Sophie Ansoborlo, Claire Freycon, Gérard Michel, Julie Berbis, Justyna Kanold, CHU Pontchaillou [Rennes], Centre d'études et de recherche sur les services de santé et la qualité de vie (CEReSS), Aix Marseille Université (AMU), Service de pédiatrie multidisciplinaire [Hôpital de la Timone Enfants - APHM], Hôpital de la Timone [CHU - APHM] (TIMONE), Institut d'hématologie et d'oncologie pédiatrique [CHU - HCL] (IHOPe), Hospices Civils de Lyon (HCL), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon, CHU Bordeaux [Bordeaux], Unité d'Hémato-Immunologie pédiatrique [Hôpital Robert Debré, Paris], Service d'Immuno-hématologie pédiatrique [Hôpital Robert Debré, Paris], Hôpital Robert Debré-Hôpital Robert Debré, CHU Strasbourg, Service d'Oncologie Pédiatrique [CHRU Nancy], Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Centre Hospitalier Universitaire de Nice (CHU Nice), Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Centre Hospitalier Universitaire de Saint-Etienne [CHU Saint-Etienne] (CHU ST-E), Centre d'Investigation Clinique - Innovation Technologique - INSERM - CHU de Grenoble (CIC-IT Grenoble (CIT803)), CHU Grenoble-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Grenoble Alpes (UGA), Centre Hospitalier Universitaire [Grenoble] (CHU), CHU Saint-Etienne, and CCSD, Accord Elsevier
Subjects: Male, Childhood leukemia, Health Status, Childhood Cancer Survivor Study, Logistic regression, Risk Assessment, school performance, Young Adult, 03 medical and health sciences, 0302 clinical medicine, Risk Factors, Humans, Medicine, childhood cancer, acute leukemia, Child, Socioeconomic status, Acute leukemia, [SDV.MHEP] Life Sciences [q-bio]/Human health and pathology, Leukemia, business.industry, Incidence, school impact, medicine.disease, 3. Good health, Survival Rate, Cross-Sectional Studies, Socioeconomic Factors, 030220 oncology & carcinogenesis, adolescent, long-term survivors, Pediatrics, Perinatology and Child Health, Cohort, Quality of Life, social impact, Female, France, business, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology, Follow-Up Studies, Forecasting, 030215 immunology, Social status, Demography
Abstract: International audience; OBJECTIVE:To evaluate the association between medical and social environmental factors and the risk of repeating a grade in childhood leukemia survivors.STUDY DESIGN:A cross-sectional study of childhood leukemia survivors, recruited through the LEA cohort (Leucémie de l'Enfant et de l'Adolescent [French Childhood Cancer Survivor Study for Leukemia]) in 2014. An adjusted logistic regression model was used to identify variables linked to repeating a grade after the diagnosis among the survivors, and the rates of repeating a grade were compared between the survivors and their siblings using a multilevel logistic regression model.RESULTS:The mean age at inclusion of the 855 participants was 16.2 ± 7.0 years, and the mean duration of follow-up from diagnosis to evaluation was 10.2 ± 6.2 years. After disease onset, 244 patients (28.5%) repeated a grade, with a median interval of 4 years (IQR, 2-8 years). Independent factors associated with repeating a grade were male sex (OR, 1.78; 95% CI, 1.21-2.60), adolescence (OR, 2.70; 95% CI, 1.63-4.48), educational support during the treatment period (OR, 3.79; 95% CI, 2.45-5.88), low parental education level (OR, 2.493; 95% CI, 1.657-3.750), and household financial difficulties (OR, 2.62; 95% CI, 1.607-4.28). Compared with siblings, survivors were at greater risk of repeating a grade (OR, 1.87; 95% CI, 1.48-2.35).CONCLUSIONS:The most vulnerable patients seemed to be adolescents and those with parents of low socioeconomic status. Improving the schooling career of leukemia survivors will require that the medical community more carefully consider the social status of patients.
Published: 2019
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9. Conventional radiography in juvenile idiopathic arthritis: Joint recommendations from the French societies for rheumatology, radiology and paediatric rheumatology

Author: Elisabeth Solau-Gervais, Linda Rossi-Semerano, Brigitte Bader-Meunier, Chantal Job Deslandre, Catherine Adamsbaum, Pauline Marteau, Cécile Gaujoux-Viala, Tu Anh Tran, Irène Lemelle, Sylvain Breton, Christelle Sordet, Julien Wipff, Pascal Pillet, Anne Lohse, Michel De Bandt, Valérie Devauchelle-Pensec, CHRU Brest - Service de Rhumatologie (CHU - BREST - Rhumato), Centre Hospitalier Régional Universitaire de Brest (CHRU Brest), AP-HP Hôpital Bicêtre (Le Kremlin-Bicêtre), Hôpital Pierre Zobda-Quitman [CHU de la Martinique], CHU de la Martinique [Fort de France], Service d'Oncologie Pédiatrique [CHRU Nancy], Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Institut Cochin (IC UM3 (UMR 8104 / U1016)), Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Université Paris Descartes - Paris 5 (UPD5), Centre Hospitalier Universitaire de Nîmes (CHU Nîmes), Cellules Souches, Plasticité Cellulaire, Médecine Régénératrice et Immunothérapies (IRMB), Université de Montpellier (UM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Hôpital Nord Franche-Comté [Hôpital de Trévenans] (HNFC), Centre hospitalier universitaire de Poitiers (CHU Poitiers), Hôpital de Hautepierre [Strasbourg], CHU de Bordeaux Pellegrin [Bordeaux], CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Hôpital Universitaire Carémeau [Nîmes] (CHU Nîmes), Lymphocyte B et Auto-immunité (LBAI), Université de Brest (UBO)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut Brestois Santé Agro Matière (IBSAM), Université de Brest (UBO), Michel, Geneviève, Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Montpellier (UM), Lymphocytes B, Autoimmunité et Immunothérapies (LBAI), Université de Brest (UBO)-Institut National de la Santé et de la Recherche Médicale (INSERM)-LabEX IGO Immunothérapie Grand Ouest, and Nantes Université (Nantes Univ)-Nantes Université (Nantes Univ)-Institut Brestois Santé Agro Matière (IBSAM)
Subjects: Male, musculoskeletal diseases, medicine.medical_specialty, Adolescent, genetic structures, [SDV]Life Sciences [q-bio], education, Arthritis, Recommendations, 03 medical and health sciences, 0302 clinical medicine, immune system diseases, Internal medicine, medicine, Humans, Radiology, Nuclear Medicine and imaging, In patient, 030212 general & internal medicine, Child, skin and connective tissue diseases, Neuroradiology, Conventional radiography, 030203 arthritis & rheumatology, medicine.diagnostic_test, business.industry, Interventional radiology, General Medicine, Juvenile idiopathic arthritis, medicine.disease, Arthritis, Juvenile, Rheumatology, 3. Good health, [SDV] Life Sciences [q-bio], Radiography, Systematic review, Musculoskeletal, Female, Radiology, business, Structural damage, Erosions, Paediatric rheumatology
Abstract: Background Juvenile idiopathic arthritis (JIA) can cause structural damage. However, data on conventional radiography (CR) in JIA are scant. Objective To provide pragmatic guidelines on CR in each non-systemic JIA subtype. Methods A multidisciplinary task force of 16 French experts (rheumatologists, paediatricians, radiologists and one patient representative) formulated research questions on CR assessments in each non-systemic JIA subtype. A systematic literature review was conducted to identify studies providing detailed information on structural joint damage. Recommendations, based on the evidence found, were evaluated using two Delphi rounds and a review by an independent committee. Results 74 original articles were included. The task force developed four principles and 31 recommendations with grades ranging from B to D. The experts felt strongly that patients should be selected for CR based on the risk of structural damage, with routine CR of the hands and feet in rheumatoid factor-positive polyarticular JIA but not in oligoarticular non-extensive JIA. Conclusion These first pragmatic recommendations on CR in JIA rely chiefly on expert opinion, given the dearth of scientific evidence. CR deserves to be viewed as a valuable tool in many situations in patients with JIA. Key Points • CR is a valuable imaging technique in selected indications. • CR is routinely recommended for peripheral joints, when damage risk is high. • CR is recommended according to the damage risk, depending on JIA subtype. • CR is not the first-line technique for imaging of the axial skeleton. Electronic supplementary material The online version of this article (10.1007/s00330-018-5304-7) contains supplementary material, which is available to authorized users.
Published: 2018
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10. Phase I study of non-pegylated liposomal doxorubicin in children with recurrent/refractory high-grade glioma

Author: Didier Frappaz, Bénédicte Devictor, Birgit Geoerger, Isabelle Aerts, Pascal Chastagner, S. Bracard, Pierre Leblond, Jean-Claude Gentet, Nicolas André, Service d'Oncologie Pédiatrique [CHRU Nancy], Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Aix-Marseille Université - Faculté de médecine (AMU MED), Aix Marseille Université (AMU), Hôpital de la Timone [CHU - APHM] (TIMONE), Département de cancérologie de l'enfant et de l'adolescent [Gustave Roussy], Institut Gustave Roussy (IGR), Institut Curie [Paris], Centre Régional de Lutte contre le Cancer Oscar Lambret [Lille] (UNICANCER/Lille), Université Lille Nord de France (COMUE)-UNICANCER, Institut d'hématologie et d'oncologie pédiatrique [CHU - HCL] (IHOPe), Hospices Civils de Lyon (HCL), Département de neuroradiologie diagnostique et thérapeutique [CHRU Nancy], Imagerie Adaptative Diagnostique et Interventionnelle (IADI), Université de Lorraine (UL)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre de Recherches en Oncologie biologique et Oncopharmacologie (CRO2), Institut National de la Santé et de la Recherche Médicale (INSERM)- Hôpital de la Timone [CHU - APHM] (TIMONE)-Aix Marseille Université (AMU), Université de Lille-UNICANCER, Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lorraine (UL), and Aix Marseille Université (AMU)- Hôpital de la Timone [CHU - APHM] (TIMONE)-Institut National de la Santé et de la Recherche Médicale (INSERM)
Subjects: Male, Oncology, Cancer Research, medicine.medical_specialty, Adolescent, Liposomal Doxorubicin, Pharmacology, Toxicology, Treatment failure, Pegylated Liposomal Doxorubicin, 03 medical and health sciences, 0302 clinical medicine, Neoplasm Recurrence, Refractory, Pharmacokinetics, Internal medicine, Humans, Medicine, Pharmacology (medical), Treatment Failure, Child, High-Grade Glioma, Antibiotics, Antineoplastic, Brain Neoplasms, business.industry, Glioma, 3. Good health, Phase i study, Doxorubicin, 030220 oncology & carcinogenesis, Liposomes, Female, Neoplasm Recurrence, Local, business, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology, 030217 neurology & neurosurgery
Abstract: International audience; PURPOSE:To determine the maximum recommended dose (RD) and pharmacokinetics of Myocet®, a non-pegylated liposomal doxorubicin, in children.METHODS:Eligible patients were children with refractory high-grade glioma who had received prior chemotherapy and radiotherapy but no anthracyclines. Cohorts of at least three patients each received escalating doses of Myocet® starting at 60 mg/m(2) at 3-week intervals, administered intravenously over 1 h, and then doses were escalated to 75 mg/m(2) corresponding to the adult RD. Periodic blood samples were collected, and plasma doxorubicin and doxorubicinol concentrations were quantified to characterise the pharmacokinetics of Myocet®.RESULTS:Between October 2010 and January 2013, 13 children aged 6-17 years were treated. In total, 27 courses were administered, at the 60 mg/m(2) dose level in seven patients without dose-limiting toxicity (DLT), and at 75 mg/m(2) in six patients of whom two experienced DLT (grade 4 neutropenia). The most common grade 3-4 toxicities reported for all courses were neutropenia (35 and 38 %, respectively), thrombocytopenia (12 and 4 %, respectively); and grade 3 vomiting, nausea, mucositis, and fever (4 % each). Mean estimates of central volume of distribution at steady state, clearance, and elimination half-life of doxorubicin were 24.8 L, 15 L/h/m(2), and 34.8 h, respectively, with a large interpatient variability.CONCLUSION:The RD of Myocet® administered every 3 weeks to paediatric patients was 60 mg/m(2). The efficacy of Myocet® in paediatric patients with high-grade glioma remains to be determined and should be studied in Phase II trials.
Published: 2015
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11. The therapeutic alliance is associated with a better therapeutic adherence in children with juvenile idiopathic arthritis: reulsts of a French Multicenter Study

Author: Devauchelle-Pensec, Valérie, Lohse, Anne, Guillemin, Francis, Solau-Gervais, Elisabeth, Rossi-Semerano, Linda, Duquesne, Agnès, Lemelle, Irène, Pillet, Pascal, Ballot, Claire, Goumy, Laurence, Tran, Tu Anh, Sparsa, Laeticia, Reumaux, Heloise, Arbault, Anaïs, Alleyrat, Camille, Michel, Geneviève, Lymphocyte B et Auto-immunité (LBAI), Université de Brest (UBO)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut Brestois Santé Agro Matière (IBSAM), Université de Brest (UBO), CHRU Brest - Service de Rhumatologie (CHU - BREST - Rhumato), Centre Hospitalier Régional Universitaire de Brest (CHRU Brest), Centre Hospitalier de Belfort (Service de Rhumatologie), CH Belfort-Montbéliard, Maladies chroniques, santé perçue, et processus d'adaptation (APEMAC), Université de Lorraine (UL), Service de rhumatologie [Poitiers], Centre hospitalier universitaire de Poitiers (CHU Poitiers), Department of Paediatrics and Paediatric Rheumatology, Bicêtre Hospital, Department of Nephrology-Rheumatology-Dermatology Pediatric, Bron, Hôpital Femme Mère Enfant [CHU - HCL] (HFME), Hospices Civils de Lyon (HCL)-Hospices Civils de Lyon (HCL), Service d'Oncologie Pédiatrique [CHRU Nancy], Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Service de Pédiatrie, Bordeaux (Pellegrin-Enfants), Service de Rhumatologie [CHRU de Besançon], Centre Hospitalier Régional Universitaire [Besançon] (CHRU Besançon), Endocrinologie pédiatrique[CHU Angers], Université d'Angers (UA)-Centre Hospitalier Universitaire d'Angers (CHU Angers), PRES Université Nantes Angers Le Mans (UNAM)-PRES Université Nantes Angers Le Mans (UNAM), Paediatrics, Nîmes, Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier)-CHU Caremeau, Nîmes-Centre Hospitalier Universitaire de Nîmes (CHU de Nîmes), Rheumatology, Mulhouse, Centre Hospitalier Emile Muller [Mulhouse] (CH E.Muller Mulhouse), Groupe Hospitalier de Territoire Haute Alsace (GHTHA)-Groupe Hospitalier de Territoire Haute Alsace (GHTHA), Pediatric Rheumatology, Lille, Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Service de Rhumatologie (CHU de Dijon), Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), CIC-Nancy, Institut Lorrain du Coeur et des Vaisseaux Louis Mathieu [Nancy]-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de Rhumatologie, Centre Hospitalier Régional Universitaire de Besançon (CHRU Besançon), Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier)-Centre Hospitalier Universitaire de Nîmes (CHU Nîmes)-Hôpital Universitaire Carémeau [Nîmes] (CHU Nîmes), Centre Hospitalier Universitaire de Nîmes (CHU Nîmes), Centre d'investigation clinique [Nancy] (CIC), and Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lorraine (UL)
Subjects: [SDV] Life Sciences [q-bio], [SDV]Life Sciences [q-bio], ComputingMilieux_MISCELLANEOUS
Abstract: International audience
Published: 2018

12. Prevalence and risk factors of iron overload after hematopoietic stem cell transplantation for childhood acute leukemia: a LEA study

Author: Sirvent , A., Auquier , P., Oudin , C., Bertrand , Y, Bohrer , S, Chastagner , P., Poirée , M., Kanold , J., Thouvenin , S, Perel , Y., Plantaz , D., Tabone , M-D, Yakouben , K., Gandemer , V., Lutz , P., Sirvent , N., Vercasson , C., Berbis , J., Chambost , H., Leverger , G., Baruchel , A., Michel , G., Centre Hospitalier Régional Universitaire [Montpellier] ( CHRU Montpellier ), Centre d'études et de recherche sur les services de santé et la qualité de vie ( CEReSS ), Aix Marseille Université ( AMU ), Hôpital de la Timone [CHU - APHM] ( TIMONE ), Service de pédiatrie, d'hématologie et d'oncologie [Hôpital de La Timone - APHM], Assistance Publique - Hôpitaux de Marseille ( APHM ) - Hôpital de la Timone [CHU - APHM] ( TIMONE ), Institut d'hématologie et d'oncologie pédiatrique [CHU - HCL] ( IHOPe ), Hospices Civils de Lyon ( HCL ), Service d'Oncologie Pédiatrique [CHRU Nancy], Centre Hospitalier Régional Universitaire de Nancy ( CHRU Nancy ), CHU Nice, CHU Clermont-Ferrand, CHU Saint-Etienne, CHU Bordeaux [Bordeaux], CHU Grenoble, Service d’oncologie hématologie pédiatrique [CHU Trousseau], Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Trousseau [APHP], Service d'hématologie et immunologie pédiatrique, Assistance publique - Hôpitaux de Paris (AP-HP)-Hôpital Robert Debré-Université Paris Diderot - Paris 7 ( UPD7 ), Université Paris Diderot - Paris 7 ( UPD7 ), CHU Pontchaillou [Rennes], and CHU Strasbourg
Subjects: [ SDV.MHEP.HEM ] Life Sciences [q-bio]/Human health and pathology/Hematology, [ SDV.IMM ] Life Sciences [q-bio]/Immunology, [ SDV.CAN ] Life Sciences [q-bio]/Cancer
Abstract: International audience; Data on post-transplant iron overload (IO) are scarce in pediatrics. We conducted a prospective multicenter cohort study (Leucémie de l'Enfant et de l'Adolescent cohort) to determine the prevalence and risk factors of IO in 384 acute leukemia survivors transplanted during childhood. Prevalence of IO (ferritin level ⩾350 ng/mL) was 42.2% (95%CI 37.2-47.2%). Factors significantly associated with IO were: 1) in univariate analysis: older age at transplant (P12.7 years and OR=5.36, 95% CI: 2.63-10.95 for age from 8.2 to 12.7 years compared to age < 4.7 years), acute myeloid leukemia (OR=3.23, 95% CI: 1.47-7.13), allogeneic graft (OR=4.34, 95% CI: 2.07-9.12 for alternative donors and OR=2.53, 95% CI: 1.2-5.33 for siblings, compared to autologous graft) and radiation-based conditioning regimen (OR=2.45, 95% CI: 1.09-5.53). Graft-versus-host disease was an additional risk factor for allogeneic graft recipients. In conclusion, IO is a frequent complication in pediatric long-term survivors after transplantation for acute leukemia, more frequently observed in older children, those transplanted from alternative donors or with graft-versus-host disease.
Published: 2017
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13. Prevalence and risk factors of iron overload after hematopoietic stem cell transplantation for childhood acute leukemia: a LEA study

Author: Sirvent , A., Auquier , P., Oudin , C., Bertrand , Y, Bohrer , S, Chastagner , P., Poirée , M., Kanold , J., Thouvenin , S, Perel , Y., Plantaz , D., Tabone , M-D, Yakouben , K., Gandemer , V., Lutz , P., Sirvent , N., Vercasson , C., Berbis , J., Chambost , H., Leverger , G., Baruchel , A., Michel , G., Centre Hospitalier Régional Universitaire [Montpellier] ( CHRU Montpellier ), Centre d'études et de recherche sur les services de santé et la qualité de vie ( CEReSS ), Aix Marseille Université ( AMU ), Hôpital de la Timone [CHU - APHM] ( TIMONE ), Service de pédiatrie, d'hématologie et d'oncologie [Hôpital de La Timone - APHM], Assistance Publique - Hôpitaux de Marseille ( APHM ) - Hôpital de la Timone [CHU - APHM] ( TIMONE ), Institut d'hématologie et d'oncologie pédiatrique [CHU - HCL] ( IHOPe ), Hospices Civils de Lyon ( HCL ), Service d'Oncologie Pédiatrique [CHRU Nancy], Centre Hospitalier Régional Universitaire de Nancy ( CHRU Nancy ), CHU Nice, CHU Clermont-Ferrand, CHU Saint-Etienne, CHU Bordeaux [Bordeaux], CHU Grenoble, Service d’oncologie hématologie pédiatrique [CHU Trousseau], Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Trousseau [APHP], Service d'hématologie et immunologie pédiatrique, Assistance publique - Hôpitaux de Paris (AP-HP)-Hôpital Robert Debré-Université Paris Diderot - Paris 7 ( UPD7 ), Université Paris Diderot - Paris 7 ( UPD7 ), CHU Pontchaillou [Rennes], and CHU Strasbourg
Subjects: [ SDV.MHEP.HEM ] Life Sciences [q-bio]/Human health and pathology/Hematology, [ SDV.IMM ] Life Sciences [q-bio]/Immunology, [ SDV.CAN ] Life Sciences [q-bio]/Cancer
Abstract: International audience; Data on post-transplant iron overload (IO) are scarce in pediatrics. We conducted a prospective multicenter cohort study (Leucémie de l'Enfant et de l'Adolescent cohort) to determine the prevalence and risk factors of IO in 384 acute leukemia survivors transplanted during childhood. Prevalence of IO (ferritin level ⩾350 ng/mL) was 42.2% (95%CI 37.2-47.2%). Factors significantly associated with IO were: 1) in univariate analysis: older age at transplant (P12.7 years and OR=5.36, 95% CI: 2.63-10.95 for age from 8.2 to 12.7 years compared to age < 4.7 years), acute myeloid leukemia (OR=3.23, 95% CI: 1.47-7.13), allogeneic graft (OR=4.34, 95% CI: 2.07-9.12 for alternative donors and OR=2.53, 95% CI: 1.2-5.33 for siblings, compared to autologous graft) and radiation-based conditioning regimen (OR=2.45, 95% CI: 1.09-5.53). Graft-versus-host disease was an additional risk factor for allogeneic graft recipients. In conclusion, IO is a frequent complication in pediatric long-term survivors after transplantation for acute leukemia, more frequently observed in older children, those transplanted from alternative donors or with graft-versus-host disease.
Published: 2017
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14. Effect of esomeprazole on the oral absorption of dasatinib in a patient with Philadelphia-positive acute lymphoblastic leukemia

Author: Pape, Elise, Michel, Delphine, Scala-Bertola, Julien, Schiestel, Thomas, Harlé, Alexandre, Bouchet, Stéphane, Contet, Audrey, Pochon, Cécile, Gambier, Nicolas, Service de Pharmacologie Clinique et Toxicologie [CHRU Nancy], Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Ingénierie Moléculaire et Physiopathologie Articulaire (IMoPA), Université de Lorraine (UL)-Centre National de la Recherche Scientifique (CNRS), Service d'Oncologie Pédiatrique [CHRU Nancy], Faculté de Pharmacie [Nancy], Université de Lorraine (UL), Centre de Recherche en Automatique de Nancy (CRAN), Centre National de la Recherche Scientifique (CNRS)-Université de Lorraine (UL), Institut de Cancérologie de Lorraine - Alexis Vautrin [Nancy] (UNICANCER/ICL), UNICANCER, and CHU Bordeaux [Bordeaux]
Subjects: hemic and lymphatic diseases, [SDV]Life Sciences [q-bio], Case Report
Abstract: International audience; We describe herein the first reported case of a drug–drug interaction between dasatinib and esomeprazole in a patient with Philadelphia-positive acute lymphoblastic leukemia leading to a decrease in dasatinib oral absorption.
Published: 2016
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15. Acute lymphoblastic leukemia in the context of RASopathies

Author: Cédric Vignal, Carine Domenech, Christian P. Kratz, Filomena Pierri, Marion Strullu, Hélène Cavé, Francoise Mechinaud, Nathalie Aladjidi, Audrey Contet, Alain Verloes, Aurélie Caye, Valère Cacheux, Jacqueline Clavel, Alice Ferster, Julie Irving, Hémopathies Myéloïdes : Cellules Souches, Modèles Pré-Cliniques et Recherche Translationnelle (UMR 1131), Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut Universitaire d'Hématologie (IUH), Université Paris Diderot - Paris 7 (UPD7)-Université Paris Diderot - Paris 7 (UPD7), AP-HP Hôpital universitaire Robert-Debré [Paris], Hôpital Pellegrin, CHU Bordeaux [Bordeaux]-Groupe hospitalier Pellegrin, Institut d'hématologie et d'oncologie pédiatrique [CHU - HCL] (IHOPe), Hospices Civils de Lyon (HCL), Service d’oncologie hématologie pédiatrique [CHU Trousseau], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Trousseau [APHP], Service d'Oncologie Pédiatrique [CHRU Nancy], Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Université de Montpellier (UM), Centre de Recherche Épidémiologie et Statistique Sorbonne Paris Cité (CRESS (U1153 / UMR_A_1125 / UMR_S_1153)), Institut National de la Recherche Agronomique (INRA)-Université Paris Diderot - Paris 7 (UPD7)-Université Paris Descartes - Paris 5 (UPD5)-Université Sorbonne Paris Cité (USPC)-Institut National de la Santé et de la Recherche Médicale (INSERM), Registre National des Cancers de l'Enfant - National Registry of Childhood Cancers [Villejuif & CHU Nancy] (RNCE), Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy)-Institut National de la Santé et de la Recherche Médicale (INSERM), Neuroprotection du Cerveau en Développement / Promoting Research Oriented Towards Early Cns Therapies (PROTECT), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Robert Debré-Université Paris Diderot - Paris 7 (UPD7)-Institut National de la Santé et de la Recherche Médicale (INSERM), Université Paris Diderot - Paris 7 (UPD7)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut National de la Recherche Agronomique (INRA)-Université Paris Descartes - Paris 5 (UPD5)-Université Sorbonne Paris Cité (USPC), 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), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service d'hématologie-immunologie-oncologie pédiatrique [CHU Trousseau], CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), and Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Robert Debré-Université Paris Diderot - Paris 7 (UPD7)-Institut National de la Santé et de la Recherche Médicale (INSERM)
Subjects: 0301 basic medicine, Oncology, Male, medicine.medical_specialty, Adolescent, Genotype, Context (language use), Protein Tyrosine Phosphatase, Non-Receptor Type 11, RASopathy, Acute lymphoblastic leukemia, PTPN11, 03 medical and health sciences, 0302 clinical medicine, Costello syndrome, Internal medicine, Genetics, medicine, Prevalence, Humans, Noonan syndrome, HRAS, SOS1, Child, RASopathies, Genetics (clinical), business.industry, Infant, Newborn, Infant, Neoplasms, Second Primary, General Medicine, Precursor Cell Lymphoblastic Leukemia-Lymphoma, medicine.disease, 3. Good health, Leukemia, 030104 developmental biology, [SDV.GEN.GH]Life Sciences [q-bio]/Genetics/Human genetics, 030220 oncology & carcinogenesis, Child, Preschool, Mutation, ras Proteins, SHP2, Female, business, SOS1 Protein
Abstract: International audience; Noonan syndrome is associated with a range of malignancies including acute lymphoblastic leukemia (ALL). However, little information is available regarding the frequency, natural history, characteristics and prognosis of ALL in Noonan syndrome or RASopathies in general. Cross-referencing data from a large prospective cohort of 1176 patients having a molecularly confirmed RASopathy with data from the French childhood cancer registry allowed us to identify ALL in 6 (0.5%) patients including 4/778 (0.5%) with a germline PTPN11 mutation and 2/94 (2.1%) with a germline SOS1 mutation. None of the patients of our series with CFC syndrome (with germline BRAF or MAP2K1/MAP2K2 mutation - n = 121) or Costello syndrome (with HRAS mutation - n = 35) had an ALL. A total of 19 Noonan-ALL were gathered by adding our patients to those of the International Berlin-Munster-Frankfurt (I-BFM) study group and previously reported patients. Strikingly, all Noonan-associated ALL were B-cell precursor ALL, and high hyperdiploidy with more than 50 chromosomes was found in the leukemia cells of 13/17 (76%) patients with available genetics data. Our data suggest that children with Noonan syndrome are at higher risk to develop ALL. Like what is observed for somatic PTPN11 mutations, NS is preferentially associated with the development of hyperdiploid ALL that will usually respond well to chemotherapy. However, Noonan syndrome patients seem to have a propensity to develop post therapy myelodysplasia that can eventually be fatal. Hence, one should be particularly cautious when treating these patients.
Published: 2016
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16. Contrast-enhanced ultrasound in fibro-lamellar hepatocellular carcinoma: a case report

Author: Michel Claudon, Damien Mandry, Marie-Agnès Galloy, Aude Bressenot, Pascal Chastagner, Sophie Branchereau, Imagerie Adaptative Diagnostique et Interventionnelle (IADI), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lorraine (UL), Service de Pathologie [CHRU Nancy], Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Service d'Oncologie Pédiatrique [CHRU Nancy], Département de chirurgie pédiatrique, and Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Bicêtre
Subjects: Oncology, medicine.medical_specialty, Pathology, 030218 nuclear medicine & medical imaging, 03 medical and health sciences, 0302 clinical medicine, Text mining, MESH: Liver Neoplasms, MESH: Child, Internal medicine, Medicine, Radiology, Nuclear Medicine and imaging, Lamellar structure, MESH: Carcinoma, Hepatocellular, MESH: Treatment Outcome, MESH: Humans, business.industry, medicine.disease, [SDV.BIBS]Life Sciences [q-bio]/Quantitative Methods [q-bio.QM], MESH: Sensitivity and Specificity, 3. Good health, 030220 oncology & carcinogenesis, Hepatocellular carcinoma, MESH: Image Enhancement, [INFO.INFO-BI]Computer Science [cs]/Bioinformatics [q-bio.QM], business, MESH: Female, MESH: Ultrasonography, Contrast-enhanced ultrasound
Abstract: International audience; A case of fibrolamellar hepatocellular carcinoma, occurring in a 13 year-old girl with the characteristics of the dynamic enhancement observed during contrast-enhanced ultrasound within the tumour and within vascular extension into the portal vein is reported. The lesion showed marked hypervascularity in the early arterial and arterial phases with irregular, tortuous vessels. During the early portal phase, centripetal filling of the tumour was observed, except in an irregular area and adjacent hypo-enhancing bands, related to a central scar, on subsequent CT and MRI images. Centrifugal wash-out led to the absence of sustained enhancement during the late phase. Similar enhancement patterns were observed within the portal tumoural thrombosis, and evaluation of its extension was easy on delayed images. Contrast-enhanced ultrasound is a useful diagnostic tool for the characterisation and evaluation of extension of such a rare malignant hepatic tumour.
Published: 2007
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17. PRKDC mutations associated with immunodeficiency, granuloma, and autoimmune regulator-dependent autoimmunity

Author: Marie Therese Zabot, Jolan E. Walter, Anne Laure Mathieu, Marie Chansel, Gillian I. Rice, Jean-Pierre de Villartay, Luigi D. Notarangelo, Christine Ménétrier-Caux, François Plenat, Nicole Fabien, Brigitte Balme, Kari C. Nadeau, Chantal Rigal, E. Helen Kemp, Annick Lim, Christophe Malcus, Yves Bertrand, Sébastien Viel, Mirjam van der Burg, Despina Moshous, Jacques Bienvenu, A. Phan, Christine Bardin, Catharina Schuetz, Thierry Walzer, Christophe Caux, Capucine Picard, Ana Delgado, David J. Chen, Manish J. Butte, Krisztian Csomos, Isabelle Rouvet, Karin Chen, Jean François Meritet, Alexandre Belot, Héloïse Reumaux, Fanny Fouyssac, Pierre Cochat, Yanick J. Crow, Estelle Verronese, Réponse immunitaire innée dans les maladies infectieuses et auto-immunes – Innate immunity in infectious and autoimmune diseases, Centre International de Recherche en Infectiologie - UMR (CIRI), École normale supérieure - Lyon (ENS Lyon)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-École normale supérieure - Lyon (ENS Lyon)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Centre de Recherche en Cancérologie de Lyon (UNICANCER/CRCL), Centre Léon Bérard [Lyon]-Université Claude Bernard Lyon 1 (UCBL), Manchester Centre for Genomic Medicine (MCGM), Manchester Academic Health Science Centre (MAHSC), University of Manchester [Manchester]-University of Manchester [Manchester]-Faculty of Biology, Medicine and Health [Manchester, UK], University of Manchester [Manchester]-Manchester University NHS Foundation Trust (MFT)-St Mary's Hospital Manchester, Service d'Oncologie Pédiatrique [CHRU Nancy], Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Institut d'hématologie et d'oncologie pédiatrique [CHU - HCL] (IHOPe), Hospices Civils de Lyon (HCL), Imagine - Institut des maladies génétiques (IMAGINE - U1163), Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM), Center for Immunology and Inflammatory Diseases [Boston, MA, USA], Harvard Medical School [Boston] (HMS), Department of Microbiology and Immunology [Stanford], Stanford Medicine, Stanford University-Stanford University, University of Texas Southwestern Medical Center, University of Utah School of Medicine [Salt Lake City], Department of Pediatrics and Adolescent Medicine, Universität Ulm - Ulm University [Ulm, Allemagne], Unité Pédiatrique [Jeanne de Flandre], Hôpital Jeanne de Flandre [Lille], Service de Pathologie [CHRU Nancy], Epidémiologie, Pharmacologie, Investigation Clinique, Information médicale, Mère-Enfant (EPICIME), Département de Pathologie [CHU Lyon-Sud - HCL], Centre Hospitalier Lyon Sud [CHU - HCL] (CHLS), Hospices Civils de Lyon (HCL)-Hospices Civils de Lyon (HCL), Erasmus University Medical Center [Rotterdam] (Erasmus MC), University of Sheffield [Sheffield], Hospices Civils de Lyon, Laboratoire d'Immunologie, Groupement Hospitalier Edouard Herriot, 5 Place d'Arsonval, F-69437, Lyon Cedex 03, France, parent, Service de Virologie [CHU Cochin], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Institut Pasteur [Paris], Centre International de Recherche en Infectiologie (CIRI), École normale supérieure de Lyon (ENS de Lyon)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-École normale supérieure de Lyon (ENS de Lyon)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Manchester Centre for Genomic Medicine [Manchester, UK] (MCGM), St Mary's Hospital Manchester-Manchester Academic Health Science Centre (MAHSC), University of Manchester [Manchester]-University of Manchester [Manchester]-Manchester University NHS Foundation Trust (MFT)-Faculty of Biology, Medicine and Health [Manchester, UK], University of Manchester [Manchester], Institut Pasteur [Paris] (IP), Centre de Recherche en Cancérologie de Lyon (CRCL), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Centre Léon Bérard [Lyon]-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Manchester Centre for Genomic Medicine, St Mary's Hospital-Central Manchester University Hospitals NHS Foundation Trust-Manchester Academic Health Sciences Centre, Centre National de la Recherche Scientifique (CNRS)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM), Department of Microbiology and Immunology, Stanford University School of Medicine [CA, USA], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Cochin [AP-HP], and Immunology
Subjects: Male, DNA End-Joining Repair, Skin Neoplasms, DCLRE1C, Autoimmunity, DNA-Activated Protein Kinase, medicine.disease_cause, DNA-Dependent Protein Kinase Catalytic Subunit, Immune tolerance, Mice, Immunology and Allergy, DNA-PKcs, B-Lymphocytes, Granuloma, tolerance, PRKDC, recombination-activating gene, V(D)J recombination, Nuclear Proteins, severe combined immunodeficiency, Autoimmune regulator, 3. Good health, [SDV.MP.VIR]Life Sciences [q-bio]/Microbiology and Parasitology/Virology, [SDV.IMM]Life Sciences [q-bio]/Immunology, Female, Adolescent, Immunology, Biology, Article, Young Adult, Th2 Cells, SDG 3 - Good Health and Well-being, medicine, Immune Tolerance, Animals, Humans, Autoantibodies, Severe combined immunodeficiency, Immunologic Deficiency Syndromes, Th1 Cells, VDJ recombination, medicine.disease, [SDV.MP.BAC]Life Sciences [q-bio]/Microbiology and Parasitology/Bacteriology, V(D)J Recombination, Gene Expression Regulation, DNA-dependent protein kinase catalytic subunit, Mutation, Cancer research, Transcription Factors
Abstract: International audience; BACKGROUND: PRKDC encodes for DNA-dependent protein kinase catalytic subunit (DNA-PKcs), a kinase that forms part of a complex (DNA-dependent protein kinase [DNA-PK]) crucial for DNA double-strand break repair and V(D)J recombination. In mice DNA-PK also interacts with the transcription factor autoimmune regulator (AIRE) to promote central T-cell tolerance. OBJECTIVE: We sought to understand the causes of an inflammatory disease with granuloma and autoimmunity associated with decreasing T- and B-cell counts over time that had been diagnosed in 2 unrelated patients. METHODS: Genetic, molecular, and functional analyses were performed to characterize an inflammatory disease evocative of a combined immunodeficiency. RESULTS: We identified PRKDC mutations in both patients. These patients exhibited a defect in DNA double-strand break repair and V(D)J recombination. Whole-blood mRNA analysis revealed a strong interferon signature. On activation, memory T~cells displayed a skewed cytokine response typical of TH2 and TH1 but not TH17. Moreover, mutated DNA-PKcs did not promote AIRE-dependent transcription of peripheral tissue antigens in~vitro. The latter defect correlated in~vivo with production of anti-calcium-sensing receptor autoantibodies, which are typically found in AIRE-deficient patients. In addition, 9 months after bone marrow transplantation, patient 1~had Hashimoto thyroiditis, suggesting that organ-specific autoimmunity might be linked to nonhematopoietic cells, such as AIRE-expressing thymic epithelial cells. CONCLUSION: Deficiency of DNA-PKcs, a key AIRE partner, can present as an inflammatory disease with organ-specific autoimmunity, suggesting a role for DNA-PKcs in regulating autoimmune responses and maintaining AIRE-dependent tolerance in human subjects.
Published: 2015
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18. Transmission of germline TP53 mutations from male carriers to female partners

Author: Myriam Bronner, Gaëlle Bougeard, Ludovic Mansuy, Aude Lamy, Laurence Brugières, François Golfier, Jean-Michel Flaman, Sophie Patrier-Sallebert, Thierry Frebourg, Christine Lasset, Stéphanie Baert-Desurmont, 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), Génomique et Médecine Personnalisée du Cancer et des Maladies Neuropsychiatriques (GPMCND), Service d'Hématologie et d'Oncologie Pédiatrique [CHRU Nancy], Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Nutrition-Génétique et Exposition aux Risques Environnementaux (NGERE), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lorraine (UL), Biostatistiques santé, Département biostatistiques et modélisation pour la santé et l'environnement [LBBE], Laboratoire de Biométrie et Biologie Evolutive - UMR 5558 (LBBE), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National de Recherche en Informatique et en Automatique (Inria)-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 Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National de Recherche en Informatique et en Automatique (Inria)-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)-Laboratoire de Biométrie et Biologie Evolutive - UMR 5558 (LBBE), Université de Lyon-Université de Lyon-Institut National de Recherche en Informatique et en Automatique (Inria)-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), Département de cancérologie de l'enfant et de l'adolescent [Gustave Roussy], Institut Gustave Roussy (IGR), Service de Chirurgie Gynécologique et Oncologique – Obstétrique [CHU Lyon Sud], Centre Hospitalier Lyon Sud [CHU - HCL] (CHLS), Hospices Civils de Lyon (HCL)-Hospices Civils de Lyon (HCL), Département de Pathologie [CHU Rouen], CHU Rouen, Normandie Université (NU)-Normandie Université (NU)-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU), Service d'Oncologie Pédiatrique [CHRU Nancy], Service de Génétique [CHRU Nancy], and Hospices Civils de Lyon (HCL)
Subjects: Adult, Male, medicine.medical_specialty, Biology, Germline, Gestational choriocarcinoma, Li-Fraumeni Syndrome, Germline mutation, Pregnancy, Genetics, medicine, Humans, Choriocarcinoma, Genetics (clinical), Germ-Line Mutation, ComputingMilieux_MISCELLANEOUS, Partial Hydatidiform Mole, Gynecology, Gestational trophoblastic disease, medicine.disease, 3. Good health, Pedigree, [SDV.GEN.GH]Life Sciences [q-bio]/Genetics/Human genetics, Li–Fraumeni syndrome, embryonic structures, Immunology, Female, Tumor Suppressor Protein p53, Pregnancy Complications, Neoplastic
Abstract: Gestational choriocarcinoma (CC) represents the most aggressive form of gestational tumours. In Europe and North America, gestational CC occurs in approximately 1/50 000 deliveries.1 We report the detection, in a gestational CC developed in a female partner of a patient with Li-Fraumeni syndrome (LFS) (MIM #151623), of the germline TP53 mutation initially detected in this LFS patient. In the French LFS series, we identified 78 fathers who were carriers of a germline TP53 mutation. Among the 213 corresponding pregnancies, we found two other cases of gestational CC in their partners. We estimate that gestational CC occurs in approximately 1% of the deliveries in female partners of TP53 mutation carriers. Gestational trophoblastic disease (GTD), which can occur after either abnormal or normal fertilisation, is characterised by the uncontrolled proliferation of trophoblastic cells normally producing the placenta. GTD includes premalignant (complete and partial hydatidiform moles) and malignant (invasive mole, gestational CC, placental-site trophoblastic and epithelioid trophoblastic tumours) lesions.1 We considered the diagnosis of LFS, a remarkable cancer predisposition characterised by the extent of tumour spectrum,2 in the family described in figure 1. The male index case had developed a cholangiocarcinoma at 37 years of age, …
Published: 2015
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19. Incidence and risk factors for cataract after haematopoietic stem cell transplantation for childhood leukaemia: an LEA study

Author: Vincent Barlogis, Claire Galambrun, Pascal Chastagner, Nicolas Sirvent, Audrey Contet, Yves Bertrand, Dominique Plantaz, Pascal Auquier, Meryl Horwitz, Julie Berbis, Claire Oudin, Justyna Kanold, Gérard Michel, Virginie Villes, Maryline Poiree, Aix Marseille Université (AMU), Service de pédiatrie, d'hématologie et d'oncologie [Hôpital de La Timone - APHM], Assistance Publique - Hôpitaux de Marseille (APHM)- Hôpital de la Timone [CHU - APHM] (TIMONE), Centre d'études et de recherche sur les services de santé et la qualité de vie (CEReSS), Service d'Oncologie Pédiatrique [CHRU Nancy], Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), 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, CHU Clermont-Ferrand, Institut d'hématologie et d'oncologie pédiatrique [CHU - HCL] (IHOPe), Hospices Civils de Lyon (HCL), CHU Grenoble, Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), and Université de Montpellier (UM)
Subjects: Adult, Male, medicine.medical_specialty, Pediatrics, Transplantation Conditioning, Adolescent, medicine.medical_treatment, haematopoietic stem cell transplantation, Graft vs Host Disease, Kaplan-Meier Estimate, Hematopoietic stem cell transplantation, Young Adult, Risk Factors, medicine, Humans, late effects, Cumulative incidence, Young adult, Busulfan, Proportional Hazards Models, Dose-Response Relationship, Drug, Radiotherapy, irradiation, business.industry, Incidence, Hematopoietic Stem Cell Transplantation, Dose fractionation, [SDV.MHEP.HEM]Life Sciences [q-bio]/Human health and pathology/Hematology, Hematology, Middle Aged, Myeloablative Agonists, Precursor Cell Lymphoblastic Leukemia-Lymphoma, Total body irradiation, Combined Modality Therapy, 3. Good health, Surgery, Transplantation, cataract, Cohort, leukaemia, Quality of Life, Female, Dose Fractionation, Radiation, business, Immunosuppressive Agents, Follow-Up Studies, medicine.drug
Abstract: International audience; Cataract was prospectively assessed by serial slip lamp tests in 271 patients included in the Leucémie Enfants Adolescents (LEA) programme, the French cohort of childhood leukaemia survivors. All had received haematopoietic stem cell transplantation (HSCT) after total body irradiation (TBI, n = 201) or busulfan-based (n = 70) myeloablative conditioning regimen. TBI was fractionated in all but six patients. The mean duration of follow-up from HSCT was 10·3 years. Cataract was observed in 113/271 patients (41·7%); 9/113 (8·1%) needed surgery. Cumulative incidence after TBI increased over time from 30% at 5 years to 70·8% and 78% at 15 and 20 years, respectively, without any plateau thereafter. The 15-year cumulative incidence was 12·5% in the Busulfan group. A higher cumulative steroid dose appeared to be a cofactor of TBI for cataract risk, in both univariate and multivariate Cox analysis. In the multivariate analysis, cataract had an impact in two quality of life domains: 'the role limitation due to physical problems' and 'the role limitation due to emotional problems'. These data suggest that with increasing follow-up, nearly all patients who receive TBI, even when fractionated, will suffer from cataract that can impact on their quality of life and that high cumulative steroid dose is a cofactor.
Published: 2015
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20. Quality of life in pediatric cancer survivors : A new contribution including goal concept

Author: Jean-Baptiste Lanfranchi, Fabienne Lemétayer, Pascal Chastagner, Maladies chroniques, santé perçue, et processus d'adaptation (APEMAC), Université Paris Descartes - Paris 5 (UPD5)-Université de Lorraine (UL), Service d'Oncologie Pédiatrique [CHRU Nancy], and Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy)
Subjects: Child quality, Operationalization, 05 social sciences, Cancer, 050109 social psychology, Life domain, medicine.disease, Pediatric cancer, 03 medical and health sciences, 0302 clinical medicine, Quality of life (healthcare), 030220 oncology & carcinogenesis, medicine, Anxiety, 0501 psychology and cognitive sciences, [SDV.SPEE]Life Sciences [q-bio]/Santé publique et épidémiologie, medicine.symptom, Psychology, Depression (differential diagnoses), Clinical psychology
Abstract: The present study focuses on quality of life in children survivors of cancer through the filter of self-regulation by goals. In this respect, the model of Dupuis and its operationalization by Missotten through the Child Quality of Life Systemic Inventory (QLSI-C©) makes it possible to identify the regulation processes (state, goal, speed, rank), in order to distinguish between children survivors of cancer and their healthy peers, as well as identifying the impact of these processes on anxiety and depression symptoms. Fifty children survivors of cancer, aged 8 to 12 years, answered three questionnaires: the QLSI-C©, the State-Trait Anxiety Inventory – Child (STAI-C), and the Child Depression Inventory (CDI). Their responses were compared with those obtained from 50 healthy children of the same age. Partial Least Squares Discriminant Analysis (PLS-DA) was used to show that the process that best differentiated the two groups pointed to a regulation through goals and priorities. Results revealed too that these processes were particularly apparent in 8 of the 20 life domains examined. Moreover, of the four processes, state is the best predictor of depressive symptoms. Children survivors of cancer do not view their lives and plans in the same manner as their healthy peers. However, their different strategies of regulating goals and priorities do not result in more depressive symptoms than in their healthy peers. The question of whether more demanding goals and priorities are necessarily a source of well-being remains to be determined.
Published: 2015
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21. Late effects in survivors of infantile acute leukemia: a study of the L.E.A program

Author: Pierre G. Lutz, Justyna Kanold, G. Michel, D Plantaz, J.-H. Dalle, S. Ducassou, Julie Berbis, Claire Oudin, S Thouvenin, Guy Leverger, Jacinthe Bonneau, Marilyne Poirée, P Chastagner, Pascal Auquier, Benoit Brethon, M.-D. Tabone, Virginie Gandemer, N Sirvent, Yves Bertrand, Z Hamidou, André Baruchel, CHU Pontchaillou [Rennes], Service de pédiatrie, d'hématologie et d'oncologie [Hôpital de La Timone - APHM], Assistance Publique - Hôpitaux de Marseille (APHM)- Hôpital de la Timone [CHU - APHM] (TIMONE), Centre d'études et de recherche sur les services de santé et la qualité de vie (CEReSS), Aix Marseille Université (AMU), Institut d'hématologie et d'oncologie pédiatrique [CHU - HCL] (IHOPe), Hospices Civils de Lyon (HCL), CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), CHU Bordeaux [Bordeaux], Service d'Oncologie Pédiatrique [CHRU Nancy], Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Unité d'Hémato-Immunologie pédiatrique [Hôpital Robert Debré, Paris], Service d'Immuno-hématologie pédiatrique [Hôpital Robert Debré, Paris], Hôpital Robert Debré-Hôpital Robert Debré, Centre Hospitalier Universitaire de Saint-Etienne [CHU Saint-Etienne] (CHU ST-E), Centre Hospitalier Universitaire de Nice (CHU Nice), CHU Grenoble, 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, CHU Clermont-Ferrand, Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), CHU Strasbourg, Service d'hématologie-immunologie-oncologie pédiatrique [CHU Trousseau], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), and CHU Saint-Etienne
Subjects: Male, medicine.medical_specialty, [SDV.CAN]Life Sciences [q-bio]/Cancer, Antineoplastic Agents, Fusion gene, 03 medical and health sciences, 0302 clinical medicine, Cancer Survivors, Internal medicine, medicine, Humans, Letter to the Editor, ComputingMilieux_MISCELLANEOUS, Acute leukemia, Hematology, business.industry, Hematopoietic Stem Cell Transplantation, Infant, Newborn, Infant, Precursor Cell Lymphoblastic Leukemia-Lymphoma, medicine.disease, 3. Good health, Lymphoma, Leukemia, Myeloid, Acute, Haematopoiesis, Leukemia, Oncology, Apoptosis, 030220 oncology & carcinogenesis, Immunology, Quality of Life, Cancer research, Female, Stem cell, business, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology, 030215 immunology
Abstract: International audience
Published: 2017
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22. Kontrastmittelsonografie bei einem fibrolamellären hepatozellulären Karzinom: Ein Fallbericht

Author: D, Mandry, A, Bressenot, M A, Galloy, P, Chastagner, S, Branchereau, M, Claudon, Imagerie Adaptative Diagnostique et Interventionnelle (IADI), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lorraine (UL), Service de Pathologie [CHRU Nancy], Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Service d'Oncologie Pédiatrique [CHRU Nancy], Département de chirurgie pédiatrique, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Bicêtre, and Felblinger, Jacques
Subjects: Carcinoma, Hepatocellular, MESH: Humans, [SDV.BIBS] Life Sciences [q-bio]/Quantitative Methods [q-bio.QM], Liver Neoplasms, Image Enhancement, Sensitivity and Specificity, [SDV.BIBS]Life Sciences [q-bio]/Quantitative Methods [q-bio.QM], MESH: Sensitivity and Specificity, Treatment Outcome, MESH: Liver Neoplasms, MESH: Child, Humans, Female, MESH: Image Enhancement, [INFO.INFO-BI]Computer Science [cs]/Bioinformatics [q-bio.QM], Child, MESH: Carcinoma, Hepatocellular, MESH: Female, Ultrasonography, MESH: Treatment Outcome, MESH: Ultrasonography, [INFO.INFO-BI] Computer Science [cs]/Bioinformatics [q-bio.QM]
Abstract: International audience; A case of fibrolamellar hepatocellular carcinoma, occurring in a 13 year-old girl with the characteristics of the dynamic enhancement observed during contrast-enhanced ultrasound within the tumour and within vascular extension into the portal vein is reported. The lesion showed marked hypervascularity in the early arterial and arterial phases with irregular, tortuous vessels. During the early portal phase, centripetal filling of the tumour was observed, except in an irregular area and adjacent hypo-enhancing bands, related to a central scar, on subsequent CT and MRI images. Centrifugal wash-out led to the absence of sustained enhancement during the late phase. Similar enhancement patterns were observed within the portal tumoural thrombosis, and evaluation of its extension was easy on delayed images. Contrast-enhanced ultrasound is a useful diagnostic tool for the characterisation and evaluation of extension of such a rare malignant hepatic tumour.
Published: 2007
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22 results on '"Service d'Oncologie Pédiatrique [CHRU Nancy]"'

1. Real-world use of defibrotide for veno-occlusive disease/sinusoidal obstruction syndrome: the DEFIFrance Registry Study

2. Cerebellar swelling after surgery for medulloblastoma with leptomeningeal dissemination in children. A case based-update

3. Complications des cellules CAR-T autres que les infections, CRS et ICANS : recommandations de la Société francophone de greffe de moelle et de thérapie cellulaire (SFGM-TC)

4. Improved outcome in children compared to adolescents and young adults after allogeneic hematopoietic stem cell transplant for acute myeloid leukemia: a retrospective study from the Francophone Society of Bone Marrow Transplantation and Cell Therapy (SFGM-TC)

5. Testosterone deficiency in men surviving childhood acute leukemia after treatment with hematopoietic stem cell transplantation or testicular radiation: an L.E.A. study

6. Rapid fully-automated assay for routine molecular diagnosis of BRAF mutations for personalized therapy of low grade gliomas

7. SFCE METRO‐01 four‐drug metronomic regimen phase II trial for pediatric extracranial tumor

8. Adolescence and Socioeconomic Factors: Key Factors in the Long-Term Impact of Leukemia on Scholastic Performance—A LEA Study

9. Conventional radiography in juvenile idiopathic arthritis: Joint recommendations from the French societies for rheumatology, radiology and paediatric rheumatology

10. Phase I study of non-pegylated liposomal doxorubicin in children with recurrent/refractory high-grade glioma

11. The therapeutic alliance is associated with a better therapeutic adherence in children with juvenile idiopathic arthritis: reulsts of a French Multicenter Study

12. Prevalence and risk factors of iron overload after hematopoietic stem cell transplantation for childhood acute leukemia: a LEA study

13. Prevalence and risk factors of iron overload after hematopoietic stem cell transplantation for childhood acute leukemia: a LEA study

14. Effect of esomeprazole on the oral absorption of dasatinib in a patient with Philadelphia-positive acute lymphoblastic leukemia

15. Acute lymphoblastic leukemia in the context of RASopathies

16. Contrast-enhanced ultrasound in fibro-lamellar hepatocellular carcinoma: a case report

17. PRKDC mutations associated with immunodeficiency, granuloma, and autoimmune regulator-dependent autoimmunity

18. Transmission of germline TP53 mutations from male carriers to female partners

19. Incidence and risk factors for cataract after haematopoietic stem cell transplantation for childhood leukaemia: an LEA study

20. Quality of life in pediatric cancer survivors : A new contribution including goal concept

21. Late effects in survivors of infantile acute leukemia: a study of the L.E.A program

22. Kontrastmittelsonografie bei einem fibrolamellären hepatozellulären Karzinom: Ein Fallbericht

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