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Neutropenia in Patients with Common Variable Immunodeficiency: a Rare Event Associated with Severe Outcome.

Authors
  • Guffroy, Aurélien1, 2
  • Mourot-Cottet, Rachel3
  • Gérard, Laurence4, 5
  • Gies, Vincent1, 2
  • Lagresle, Chantal6
  • Pouliet, Aurore7
  • Nitschké, Patrick8
  • Hanein, Sylvain9
  • Bienvenu, Boris10
  • Chanet, Valérie11
  • Donadieu, Jean12
  • Gardembas, Martine13
  • Karmochkine, Marina14
  • Nove-Josserand, Raphaele15
  • Martin, Thierry1, 2
  • Poindron, Vincent1
  • Soulas-Sprauel, Pauline1, 2
  • Rieux-Laucat, Fréderic16
  • Fieschi, Claire4, 5
  • Oksenhendler, Eric4, 5
  • And 2 more
  • 1 Department of Clinical Immunology and Internal Medicine, Strasbourg University Hospital, National Reference Center for Autoimmune Diseases, Strasbourg, France. , (France)
  • 2 CNRS UPR 3572 Immunopathology and Therapeutic Chemistry/Laboratory of Excellence Medalis, Institute of Molecular and Cellular Biology (IBMC), Strasbourg, France. , (France)
  • 3 Department of Internal Medicine B, Strasbourg University Hospital, Strasbourg, France. , (France)
  • 4 Department of Clinical Immunology, Saint-Louis Hospital, Assistance Publique Hôpitaux de Paris (APHP), Paris, France. , (France)
  • 5 EA3518, Université Paris Diderot Paris 7, Paris, France. , (France)
  • 6 INSERM UMR 1163 Human Lympho Hematopopoiesis Laboratory, Paris Descartes Sorbonne Paris Cite University, Imagine Institute, Paris, France. , (France)
  • 7 INSERM UMR 1163 Bioinformatic Platform, Paris Descartes Sorbonne Paris Cite University, Imagine Institute, Paris, France. , (France)
  • 8 INSERM UMR 1163 Genomic Platform, Paris Descartes Sorbonne Paris Cite University, Imagine Institute, Paris, France. , (France)
  • 9 INSERM UMR 1163 Translational Genetic Platform, Paris Descartes Sorbonne Paris Cite University, Imagine Institute, Paris, France. , (France)
  • 10 Department of Internal Medicine, Caen University Hospital, Caen, France. , (France)
  • 11 Department of Internal Medicine, Cabinet de Médecine Interne et Maladies Infectieuses, Pôle Santé République, Clermont-Ferrand, France. , (France)
  • 12 Department of Pediatric Hematology and Oncology, Hôpital d'enfants Armand-Trousseau, Paris University Hospital, Paris, France. , (France)
  • 13 Department of Hematology, Angers University Hospital, Angers, France. , (France)
  • 14 Department of Clinical Immunology, European Hospital Georges Pompidou, Paris University Hospital, Paris, France. , (France)
  • 15 Department of Internal Medicine, Lyon University Hospital, Pierre-Bénite, France. , (France)
  • 16 INSERM UMR 1163 Immunogenetics of pediatric autoimmune diseases laboratory, Paris Descartes Sorbonne Paris Cite University, Imagine Institute, Paris, France. , (France)
  • 17 Department of Clinical Immunology and Internal Medicine, Strasbourg University Hospital, National Reference Center for Autoimmune Diseases, Strasbourg, France. [email protected] , (France)
  • 18 CNRS UPR 3572 Immunopathology and Therapeutic Chemistry/Laboratory of Excellence Medalis, Institute of Molecular and Cellular Biology (IBMC), Strasbourg, France. [email protected] , (France)
Type
Published Article
Journal
Journal of Clinical Immunology
Publisher
Springer-Verlag
Publication Date
Oct 01, 2017
Volume
37
Issue
7
Pages
715–726
Identifiers
DOI: 10.1007/s10875-017-0434-2
PMID: 28842786
Source
Medline
Keywords
License
Unknown

Abstract

Neutropenia is generally associated with another cytopenia and presumably of autoimmune origin during CVID. In the DEFI study, neutropenia is coupled with more severe clinical outcomes. It appears as an "alarm bell" considering patients' presentation and the high rate of deaths. Whole exome sequencing diagnosis should improve management.

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