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1. Pre-Transition Consultations for Adolescents With Chronic Conditions in a General Pediatric Hospital: Lessons Learned

Author

Enora Le Roux, Paul Jacquin, Agnès Dumas, Loïc Passini, Maxime Morsa, Sophie Guilmin-Crepon, Margaux Gastaldi, Corinne Alberti, Céline Gabarro, and H. Mellerio

Subjects
medicine.medical_specialty, business.industry, Family medicine, Pediatric hospital, medicine, business
Abstract

The objective of the study was to assess the clinically relevant features of the pre-transition consultation (PTC) offered in AD’venir transition unit (R.Debré hospital, Paris) to all adolescents with a chronic condition.PTCs include a face-to-face interview with pediatricians trained in adolescent medicine, exploring the past (disease history), present (daily life, Treatment Burden Questionnaire, family/peer relationships, school, hobbies, sexuality, drugs) and future (global life project, transition, Good2Go questionnaire). Twenty-seven PTC recordings were qualitatively analyzed (girls=56%/median age=17.7yrs) within a multidisciplinary group (clinicians/sociologists/psychologist/public health researchers). Respectively 6-months and 2-years after PTC, benefits of PTC were assessed in referent healthcare providers (questionnaire) and in adolescents (phone interview). PTCs were a form of training for adult care, adolescents meeting a practitioner alone often for the first time. Naming their chronic condition was frequently difficult. All complained of limitations experienced in their social life (notably diet, tiredness, laboratory/medical appointments), but not treatment itself. Most were willing to talk about sexuality. Feelings concerning transition were various, with poor representations of adult healthcare. Transfer was often unplanned, but this did not influence transition readiness. After PTC, healthcare providers often changed their practices. Two years later, transition was successful for most of adolescents. ConclusionThe PTC is a relevant, easily implemented tool to help empower adolescents and customize their transition preparation within a holistic approach. A dedicated and long consultation, with an external/non-prescriber practitioner, including a physical examination are key factors promoting the establishment of trust, sharing of intimate issues and the assessment of potential barriers to transition.

Published
2021
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2. Adult T-cell acute lymphoblastic leukemias with IL7R pathway mutations are slow-responders who do not benefit from allogeneic stem-cell transplantation

Author

Sylvain Chantepie, Cécile Fourrage, Françoise Huguet, Patrice Chevallier, Elizabeth Macintyre, Hervé Dombret, Nicolas Boissel, Emmanuelle Tavernier, Véronique Lhéritier, Stéphane Leprêtre, Loïc Passini, Vahid Asnafi, Sébastien Maury, Martine Escoffre, Carlos Graux, Ludovic Lhermitte, Emmanuel Raffoux, Patrick Villarese, Norbert Ifrah, Yves Chalandon, Thibaut Leguay, Thorsten Braun, Xavier Thomas, Mathilde Hunault, Rathana Kim, Florian Thonier, Aurore Touzart, Institut Necker Enfants-Malades (INEM - UM 111 (UMR 8253 / U1151)), Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université Paris Cité (UPCité), Recherche clinique appliquée à l'hématologie (URP_3518), Université Paris Cité (UPCité), Service d'hématologie [CHU Bordeaux], CHU Bordeaux [Bordeaux]-Hôpital Haut-Lévêque [CHU Bordeaux], CHU Bordeaux [Bordeaux], Service d’Hématologie [Centre Hospitalier Lyon Sud - HCL], Centre Hospitalier Lyon Sud [CHU - HCL] (CHLS), Hospices Civils de Lyon (HCL)-Hospices Civils de Lyon (HCL), Service d'hématologie adulte [Hôpital de Saint Louis], 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), Service Hématologie - IUCT-Oncopole [CHU Toulouse], Pôle Biologie [CHU Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Pôle IUCT [CHU Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Plateforme de Bioinformatique [Paris] (Fondation Imagine), Innate Immunity and Immunotherapy (CRCINA-ÉQUIPE 7), Centre de Recherche en Cancérologie et Immunologie Nantes-Angers (CRCINA), Université d'Angers (UA)-Université de Nantes (UN)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Centre hospitalier universitaire de Nantes (CHU Nantes)-Université d'Angers (UA)-Université de Nantes (UN)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Centre hospitalier universitaire de Nantes (CHU Nantes), Service d'hématologie [Angers], Centre Hospitalier Universitaire d'Angers (CHU Angers), PRES Université Nantes Angers Le Mans (UNAM)-PRES Université Nantes Angers Le Mans (UNAM), Génomique et Médecine Personnalisée du Cancer et des Maladies Neuropsychiatriques (GPMCND), Université de Rouen Normandie (UNIROUEN), Normandie Université (NU)-Normandie Université (NU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Université d'Angers (UA)-Université de Nantes (UN)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Centre hospitalier universitaire de Nantes (CHU Nantes), Service d'Hématologie [Nantes], Centre hospitalier universitaire de Nantes (CHU Nantes), Institut Universitaire d'Hématologie (IUH), Université Paris Diderot - Paris 7 (UPD7), Service d'hématologie biologique [Avicenne], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Avicenne [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Sorbonne Paris Nord, Groupe de recherche sur la leucémie lymphoblastique aiguë chez l'adulte [Lyon] (CHU HCL - CHLS), Institut d'Hématologie de Basse-Normandie (IHBN), 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)-Centre Régional de Lutte contre le Cancer François Baclesse [Caen] (UNICANCER/CRLC), Normandie Université (NU)-UNICANCER-Tumorothèque de Caen Basse-Normandie (TCBN)-UNICANCER, Laboratoire d’Hématologie [CHU Henri-Mondor - APHP], CHU Henri Mondor, CHU Pontchaillou [Rennes], Institut de Cancérologie de la Loire Lucien Neuwirth, Centre Hospitalier Universitaire de Saint-Etienne (CHU de Saint-Etienne), Université de Genève = University of Geneva (UNIGE), Swiss Group for Clinical Cancer Research [Bern, Switzerland], CHU Dinant-Godinne UCL Namur [Yvoir, Belgique], We thank the Swiss State Secretariat for Education, Research and Innovation (SERI) Switzerland for support. We thank Force Hemato and Association pour la Recherche contre le Cancer (ARC) for support., Bernardo, Elizabeth, Service d'Hématologie [IUCT Toulouse], Université Fédérale Toulouse Midi-Pyrénées-Institut Universitaire du Cancer de Toulouse - Oncopole (IUCT Oncopole - UMR 1037), Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-CHU Toulouse [Toulouse]-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-CHU Toulouse [Toulouse]-Institut National de la Santé et de la Recherche Médicale (INSERM), Laboratoire d'Hematologie [CHU Pontchaillou, Rennes] (Pôle de Biologie), Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-CHU Pontchaillou [Rennes], University of Geneva [Switzerland], Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université de Paris (UP), Université de Paris (UP), 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)-CHU Toulouse [Toulouse]-Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Institut National de la Santé et de la Recherche Médicale (INSERM), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Nantes - UFR de Médecine et des Techniques Médicales (UFR MEDECINE), Université de Nantes (UN)-Université de Nantes (UN)-Centre hospitalier universitaire de Nantes (CHU Nantes)-Centre National de la Recherche Scientifique (CNRS)-Université d'Angers (UA)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Nantes - UFR de Médecine et des Techniques Médicales (UFR MEDECINE), Université de Nantes (UN)-Université de Nantes (UN)-Centre hospitalier universitaire de Nantes (CHU Nantes)-Centre National de la Recherche Scientifique (CNRS)-Université d'Angers (UA), Centre Régional de Lutte contre le Cancer François Baclesse [Caen] (UNICANCER/CRLC), UNICANCER-Tumorothèque de Caen Basse-Normandie (TCBN)-Normandie Université (NU)-UNICANCER-Tumorothèque de Caen Basse-Normandie (TCBN)-Normandie Université (NU)-CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN)-Université de Caen Normandie (UNICAEN), Normandie Université (NU), UCL - (MGD) Service d'hématologie, and UCL - SSS/IREC/MONT - Pôle Mont Godinne

Subjects
0301 basic medicine, Oncology, Male, Cancer Research, Neoplasm, Residual, medicine.medical_treatment, Hematopoietic stem cell transplantation, medicine.disease_cause, Precursor T-Cell Lymphoblastic Leukemia-Lymphoma, 0302 clinical medicine, Precursor T-Cell Lymphoblastic Leukemia-Lymphoma/genetics/pathology/therapy, Receptors, Hematopoietic Stem Cell Transplantation/mortality, ddc:616, Mutation, Hematopoietic Stem Cell Transplantation, Hematology, Middle Aged, Prognosis, 3. Good health, Gene Expression Regulation, Neoplastic, Survival Rate, 030220 oncology & carcinogenesis, Tumor/genetics, Residual/genetics/pathology/therapy, Female, Stem cell, Adult, Homologous, medicine.medical_specialty, Adolescent, [SDV.CAN]Life Sciences [q-bio]/Cancer, 03 medical and health sciences, Young Adult, [SDV.CAN] Life Sciences [q-bio]/Cancer, Internal medicine, medicine, Biomarkers, Tumor, Transplantation, Homologous, Humans, Clinical significance, Interleukin-7 receptor, Chemotherapy, Neoplastic, Transplantation, Receptors, Interleukin-7, business.industry, Minimal residual disease, 030104 developmental biology, Gene Expression Regulation, Interleukin-7/genetics, Neoplasm, business, Biomarkers, Follow-Up Studies
Abstract

International audience; The prognostic value of IL7-receptor pathway (IL7Rp) mutations in T-cell acute lymphoblastic leukemia (T-ALL) remains unclear. We performed a comprehensive study of 200 adult patients with TALL included in the GRAALL2003/2005 protocols to address the clinical significance of IL7Rp mutations. Next-generation sequencing of the IL7Rp (IL7R/JAK1/ JAK3/STAT5B) revealed that IL7Rp mutations were frequent in adult TALL (28%) particularly in immature/early T-cell progenitor (ETP)-ALL. They were associated with mutations of NOTCH-pathway, PHF6, and PRC2 components but not with K/NRAS. IL7Rp mutated (IL7Rp mut) TALL were slow-responders, with a high rate of M2/M3 day-8 marrow compared with IL7Rp non-mutated (IL7Rp WT) TALL (p = 0.002) and minimal residual disease positivity at 6-weeks (MRD1) (p = 0.008) but no difference in MRD2 positivity at 12-weeks. Despite this, no adverse prognosis was evidenced when censored for allogeneic hematopoietic stem cell transplantation (HSCT). In time-dependent analysis, HSCT did not benefit IL7Rp mut patients whereas it was of marked benefit to IL7Rp WT cases. IL7Rp-mutations identify a subgroup of slow-responder T-ALLs which benefit from post-induction chemotherapy regimens but not from HSCT. Our data suggest that prior knowledge of the mutation status of IL7Rp may influence HSCT decision and help to guide therapy reduction.

Published
2020
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