Research ArticleHematologyImmunology Open Access | 10.1172/JCI169994

Marta Benavides-Nieto,1,2,3 Frédéric Adam,4 Emmanuel Martin,5 Charlotte Boussard,1,6,7 Chantal Lagresle-Peyrou,8,9 Isabelle Callebaut,10 Alexandre Kauskot,4 Christelle Repérant,4 Miao Feng,4 Jean-Claude Bordet,11 Martin Castelle,6 Guillaume Morelle,1,6 Chantal Brouzes,12 Mohammed Zarhrate,13 Patricia Panikulam,1,14 Nathalie Lambert,15 Capucine Picard,1,5,15,16 Damien Bodet,17 Jérémie Rouger-Gaudichon,17 Patrick Revy,1,2 Jean-Pierre de Villartay,1,2 and Despina Moshous1,2,6,16

1Université Paris Cité, Paris, France.

2Imagine Institute, Laboratory of Genome Dynamics in the Immune System, Equipe Labellisée Ligue Contre le Cancer, Ligue 2023, INSERM UMR 1163, Paris, France.

3General Pediatrics–Infectious Diseases and Internal Medicine, Hôpital Robert Debré, Assistance Publique-Hôpitaux de Paris (AP-HP) Nord, Paris, France.

4INSERM UMR S 1176, Laboratory for Hemostasis, Inflammation and Thrombosis (HITh), Université Paris-Saclay, Le Kremlin-Bicêtre, France.

5Laboratory Lymphocyte Activation and Susceptibility to EBV infection, INSERM UMR 1163, Imagine Institute, Paris, France.

6Pediatric Immunology, Hematology and Rheumatology, Necker-Enfants Malades University Hospital, AP-HP, Paris, France.

7Laboratory Immunogenetics of Pediatric Autoimmune Diseases, INSERM UMR 1163, Imagine Institute, Paris, France.

8Biotherapy Clinical Investigation Center, AP-HP, Paris, France.

9Laboratory Human Lymphohematopoiesis, INSERM UMR 1163, Imagine Institute, Paris, France.

10Sorbonne University, Muséum National d’Histoire Naturelle, UMR CNRS 7590, Institut de Minéralogie, de Physique des Matériaux et de Cosmochimie, IMPMC, Paris, France.

11Laboratoire d’Hémostase, Centre de Biologie Est, Hospices Civils de Lyon, Bron, France.

12Laboratory of Onco-Hematology, Necker-Enfants Malades University Hospital, AP-HP, Paris, France, and INSERM U1151, Institut Necker-Enfants Malades, Paris, France.

13Genomics Core Facility, Institut Imagine-Structure Fédérative de Recherche Necker, INSERM U1163 and INSERM US24/CNRS UAR3633, Paris Descartes Sorbonne Paris Cité University, Paris, France.

14Laboratory “Molecular basis of altered immune homeostasis,” INSERM UMR 1163, Imagine Institute, Paris, France.

15Study Center for Primary Immunodeficiencies, Necker-Enfants Malades University Hospital, AP-HP, Paris, France.

16Centre de Référence des Déficits Immunitaires Héréditaires (CEREDIH), Necker-Enfants Malades University Hospital, AP-HP, Paris, France.

17CHU de Caen Normandie, Onco-Immunohématologie Pédiatrique, Caen, France.

Address correspondence to: Despina Moshous, Unité d’Immunologie, Hématologie et Rhumatologie Pédiatriques (UIHR), Hôpital Necker-Enfants Malades, 149, Rue de Sèvres, F-75015 Paris, France, or INSERM UMR1163, Laboratory Genome Dynamics in the Immune System, Imagine Institute, 24 boulevard du Montparnasse, 75015 Paris, France. Phone: 33.1.44.49.48.23; Email: despina.moshous@inserm.fr.

Authorship note: MBN and FA are co–first authors and contributed equally to this work. EM and C Boussard contributed equally to this work.

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1Université Paris Cité, Paris, France.

2Imagine Institute, Laboratory of Genome Dynamics in the Immune System, Equipe Labellisée Ligue Contre le Cancer, Ligue 2023, INSERM UMR 1163, Paris, France.

3General Pediatrics–Infectious Diseases and Internal Medicine, Hôpital Robert Debré, Assistance Publique-Hôpitaux de Paris (AP-HP) Nord, Paris, France.

4INSERM UMR S 1176, Laboratory for Hemostasis, Inflammation and Thrombosis (HITh), Université Paris-Saclay, Le Kremlin-Bicêtre, France.

5Laboratory Lymphocyte Activation and Susceptibility to EBV infection, INSERM UMR 1163, Imagine Institute, Paris, France.

6Pediatric Immunology, Hematology and Rheumatology, Necker-Enfants Malades University Hospital, AP-HP, Paris, France.

7Laboratory Immunogenetics of Pediatric Autoimmune Diseases, INSERM UMR 1163, Imagine Institute, Paris, France.

8Biotherapy Clinical Investigation Center, AP-HP, Paris, France.

9Laboratory Human Lymphohematopoiesis, INSERM UMR 1163, Imagine Institute, Paris, France.

10Sorbonne University, Muséum National d’Histoire Naturelle, UMR CNRS 7590, Institut de Minéralogie, de Physique des Matériaux et de Cosmochimie, IMPMC, Paris, France.

11Laboratoire d’Hémostase, Centre de Biologie Est, Hospices Civils de Lyon, Bron, France.

12Laboratory of Onco-Hematology, Necker-Enfants Malades University Hospital, AP-HP, Paris, France, and INSERM U1151, Institut Necker-Enfants Malades, Paris, France.

13Genomics Core Facility, Institut Imagine-Structure Fédérative de Recherche Necker, INSERM U1163 and INSERM US24/CNRS UAR3633, Paris Descartes Sorbonne Paris Cité University, Paris, France.

14Laboratory “Molecular basis of altered immune homeostasis,” INSERM UMR 1163, Imagine Institute, Paris, France.

15Study Center for Primary Immunodeficiencies, Necker-Enfants Malades University Hospital, AP-HP, Paris, France.

16Centre de Référence des Déficits Immunitaires Héréditaires (CEREDIH), Necker-Enfants Malades University Hospital, AP-HP, Paris, France.

17CHU de Caen Normandie, Onco-Immunohématologie Pédiatrique, Caen, France.

Address correspondence to: Despina Moshous, Unité d’Immunologie, Hématologie et Rhumatologie Pédiatriques (UIHR), Hôpital Necker-Enfants Malades, 149, Rue de Sèvres, F-75015 Paris, France, or INSERM UMR1163, Laboratory Genome Dynamics in the Immune System, Imagine Institute, 24 boulevard du Montparnasse, 75015 Paris, France. Phone: 33.1.44.49.48.23; Email: despina.moshous@inserm.fr.

Authorship note: MBN and FA are co–first authors and contributed equally to this work. EM and C Boussard contributed equally to this work.

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1Université Paris Cité, Paris, France.

2Imagine Institute, Laboratory of Genome Dynamics in the Immune System, Equipe Labellisée Ligue Contre le Cancer, Ligue 2023, INSERM UMR 1163, Paris, France.

3General Pediatrics–Infectious Diseases and Internal Medicine, Hôpital Robert Debré, Assistance Publique-Hôpitaux de Paris (AP-HP) Nord, Paris, France.

4INSERM UMR S 1176, Laboratory for Hemostasis, Inflammation and Thrombosis (HITh), Université Paris-Saclay, Le Kremlin-Bicêtre, France.

5Laboratory Lymphocyte Activation and Susceptibility to EBV infection, INSERM UMR 1163, Imagine Institute, Paris, France.

6Pediatric Immunology, Hematology and Rheumatology, Necker-Enfants Malades University Hospital, AP-HP, Paris, France.

7Laboratory Immunogenetics of Pediatric Autoimmune Diseases, INSERM UMR 1163, Imagine Institute, Paris, France.

8Biotherapy Clinical Investigation Center, AP-HP, Paris, France.

9Laboratory Human Lymphohematopoiesis, INSERM UMR 1163, Imagine Institute, Paris, France.

10Sorbonne University, Muséum National d’Histoire Naturelle, UMR CNRS 7590, Institut de Minéralogie, de Physique des Matériaux et de Cosmochimie, IMPMC, Paris, France.

11Laboratoire d’Hémostase, Centre de Biologie Est, Hospices Civils de Lyon, Bron, France.

12Laboratory of Onco-Hematology, Necker-Enfants Malades University Hospital, AP-HP, Paris, France, and INSERM U1151, Institut Necker-Enfants Malades, Paris, France.

13Genomics Core Facility, Institut Imagine-Structure Fédérative de Recherche Necker, INSERM U1163 and INSERM US24/CNRS UAR3633, Paris Descartes Sorbonne Paris Cité University, Paris, France.

14Laboratory “Molecular basis of altered immune homeostasis,” INSERM UMR 1163, Imagine Institute, Paris, France.

15Study Center for Primary Immunodeficiencies, Necker-Enfants Malades University Hospital, AP-HP, Paris, France.

16Centre de Référence des Déficits Immunitaires Héréditaires (CEREDIH), Necker-Enfants Malades University Hospital, AP-HP, Paris, France.

17CHU de Caen Normandie, Onco-Immunohématologie Pédiatrique, Caen, France.

Address correspondence to: Despina Moshous, Unité d’Immunologie, Hématologie et Rhumatologie Pédiatriques (UIHR), Hôpital Necker-Enfants Malades, 149, Rue de Sèvres, F-75015 Paris, France, or INSERM UMR1163, Laboratory Genome Dynamics in the Immune System, Imagine Institute, 24 boulevard du Montparnasse, 75015 Paris, France. Phone: 33.1.44.49.48.23; Email: despina.moshous@inserm.fr.

Authorship note: MBN and FA are co–first authors and contributed equally to this work. EM and C Boussard contributed equally to this work.

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1Université Paris Cité, Paris, France.

2Imagine Institute, Laboratory of Genome Dynamics in the Immune System, Equipe Labellisée Ligue Contre le Cancer, Ligue 2023, INSERM UMR 1163, Paris, France.

3General Pediatrics–Infectious Diseases and Internal Medicine, Hôpital Robert Debré, Assistance Publique-Hôpitaux de Paris (AP-HP) Nord, Paris, France.

4INSERM UMR S 1176, Laboratory for Hemostasis, Inflammation and Thrombosis (HITh), Université Paris-Saclay, Le Kremlin-Bicêtre, France.

5Laboratory Lymphocyte Activation and Susceptibility to EBV infection, INSERM UMR 1163, Imagine Institute, Paris, France.

6Pediatric Immunology, Hematology and Rheumatology, Necker-Enfants Malades University Hospital, AP-HP, Paris, France.

7Laboratory Immunogenetics of Pediatric Autoimmune Diseases, INSERM UMR 1163, Imagine Institute, Paris, France.

8Biotherapy Clinical Investigation Center, AP-HP, Paris, France.

9Laboratory Human Lymphohematopoiesis, INSERM UMR 1163, Imagine Institute, Paris, France.

10Sorbonne University, Muséum National d’Histoire Naturelle, UMR CNRS 7590, Institut de Minéralogie, de Physique des Matériaux et de Cosmochimie, IMPMC, Paris, France.

11Laboratoire d’Hémostase, Centre de Biologie Est, Hospices Civils de Lyon, Bron, France.

12Laboratory of Onco-Hematology, Necker-Enfants Malades University Hospital, AP-HP, Paris, France, and INSERM U1151, Institut Necker-Enfants Malades, Paris, France.

13Genomics Core Facility, Institut Imagine-Structure Fédérative de Recherche Necker, INSERM U1163 and INSERM US24/CNRS UAR3633, Paris Descartes Sorbonne Paris Cité University, Paris, France.

14Laboratory “Molecular basis of altered immune homeostasis,” INSERM UMR 1163, Imagine Institute, Paris, France.

15Study Center for Primary Immunodeficiencies, Necker-Enfants Malades University Hospital, AP-HP, Paris, France.

16Centre de Référence des Déficits Immunitaires Héréditaires (CEREDIH), Necker-Enfants Malades University Hospital, AP-HP, Paris, France.

17CHU de Caen Normandie, Onco-Immunohématologie Pédiatrique, Caen, France.

Address correspondence to: Despina Moshous, Unité d’Immunologie, Hématologie et Rhumatologie Pédiatriques (UIHR), Hôpital Necker-Enfants Malades, 149, Rue de Sèvres, F-75015 Paris, France, or INSERM UMR1163, Laboratory Genome Dynamics in the Immune System, Imagine Institute, 24 boulevard du Montparnasse, 75015 Paris, France. Phone: 33.1.44.49.48.23; Email: despina.moshous@inserm.fr.

Authorship note: MBN and FA are co–first authors and contributed equally to this work. EM and C Boussard contributed equally to this work.

Find articles by Boussard, C. in: JCI | PubMed | Google Scholar

1Université Paris Cité, Paris, France.

2Imagine Institute, Laboratory of Genome Dynamics in the Immune System, Equipe Labellisée Ligue Contre le Cancer, Ligue 2023, INSERM UMR 1163, Paris, France.

3General Pediatrics–Infectious Diseases and Internal Medicine, Hôpital Robert Debré, Assistance Publique-Hôpitaux de Paris (AP-HP) Nord, Paris, France.

4INSERM UMR S 1176, Laboratory for Hemostasis, Inflammation and Thrombosis (HITh), Université Paris-Saclay, Le Kremlin-Bicêtre, France.

5Laboratory Lymphocyte Activation and Susceptibility to EBV infection, INSERM UMR 1163, Imagine Institute, Paris, France.

6Pediatric Immunology, Hematology and Rheumatology, Necker-Enfants Malades University Hospital, AP-HP, Paris, France.

7Laboratory Immunogenetics of Pediatric Autoimmune Diseases, INSERM UMR 1163, Imagine Institute, Paris, France.

8Biotherapy Clinical Investigation Center, AP-HP, Paris, France.

9Laboratory Human Lymphohematopoiesis, INSERM UMR 1163, Imagine Institute, Paris, France.

10Sorbonne University, Muséum National d’Histoire Naturelle, UMR CNRS 7590, Institut de Minéralogie, de Physique des Matériaux et de Cosmochimie, IMPMC, Paris, France.

11Laboratoire d’Hémostase, Centre de Biologie Est, Hospices Civils de Lyon, Bron, France.

12Laboratory of Onco-Hematology, Necker-Enfants Malades University Hospital, AP-HP, Paris, France, and INSERM U1151, Institut Necker-Enfants Malades, Paris, France.

13Genomics Core Facility, Institut Imagine-Structure Fédérative de Recherche Necker, INSERM U1163 and INSERM US24/CNRS UAR3633, Paris Descartes Sorbonne Paris Cité University, Paris, France.

14Laboratory “Molecular basis of altered immune homeostasis,” INSERM UMR 1163, Imagine Institute, Paris, France.

15Study Center for Primary Immunodeficiencies, Necker-Enfants Malades University Hospital, AP-HP, Paris, France.

16Centre de Référence des Déficits Immunitaires Héréditaires (CEREDIH), Necker-Enfants Malades University Hospital, AP-HP, Paris, France.

17CHU de Caen Normandie, Onco-Immunohématologie Pédiatrique, Caen, France.

Address correspondence to: Despina Moshous, Unité d’Immunologie, Hématologie et Rhumatologie Pédiatriques (UIHR), Hôpital Necker-Enfants Malades, 149, Rue de Sèvres, F-75015 Paris, France, or INSERM UMR1163, Laboratory Genome Dynamics in the Immune System, Imagine Institute, 24 boulevard du Montparnasse, 75015 Paris, France. Phone: 33.1.44.49.48.23; Email: despina.moshous@inserm.fr.

Authorship note: MBN and FA are co–first authors and contributed equally to this work. EM and C Boussard contributed equally to this work.

Find articles by Lagresle-Peyrou, C. in: JCI | PubMed | Google Scholar

1Université Paris Cité, Paris, France.

2Imagine Institute, Laboratory of Genome Dynamics in the Immune System, Equipe Labellisée Ligue Contre le Cancer, Ligue 2023, INSERM UMR 1163, Paris, France.

3General Pediatrics–Infectious Diseases and Internal Medicine, Hôpital Robert Debré, Assistance Publique-Hôpitaux de Paris (AP-HP) Nord, Paris, France.

4INSERM UMR S 1176, Laboratory for Hemostasis, Inflammation and Thrombosis (HITh), Université Paris-Saclay, Le Kremlin-Bicêtre, France.

5Laboratory Lymphocyte Activation and Susceptibility to EBV infection, INSERM UMR 1163, Imagine Institute, Paris, France.

6Pediatric Immunology, Hematology and Rheumatology, Necker-Enfants Malades University Hospital, AP-HP, Paris, France.

7Laboratory Immunogenetics of Pediatric Autoimmune Diseases, INSERM UMR 1163, Imagine Institute, Paris, France.

8Biotherapy Clinical Investigation Center, AP-HP, Paris, France.

9Laboratory Human Lymphohematopoiesis, INSERM UMR 1163, Imagine Institute, Paris, France.

10Sorbonne University, Muséum National d’Histoire Naturelle, UMR CNRS 7590, Institut de Minéralogie, de Physique des Matériaux et de Cosmochimie, IMPMC, Paris, France.

11Laboratoire d’Hémostase, Centre de Biologie Est, Hospices Civils de Lyon, Bron, France.

12Laboratory of Onco-Hematology, Necker-Enfants Malades University Hospital, AP-HP, Paris, France, and INSERM U1151, Institut Necker-Enfants Malades, Paris, France.

13Genomics Core Facility, Institut Imagine-Structure Fédérative de Recherche Necker, INSERM U1163 and INSERM US24/CNRS UAR3633, Paris Descartes Sorbonne Paris Cité University, Paris, France.

14Laboratory “Molecular basis of altered immune homeostasis,” INSERM UMR 1163, Imagine Institute, Paris, France.

15Study Center for Primary Immunodeficiencies, Necker-Enfants Malades University Hospital, AP-HP, Paris, France.

16Centre de Référence des Déficits Immunitaires Héréditaires (CEREDIH), Necker-Enfants Malades University Hospital, AP-HP, Paris, France.

17CHU de Caen Normandie, Onco-Immunohématologie Pédiatrique, Caen, France.

Address correspondence to: Despina Moshous, Unité d’Immunologie, Hématologie et Rhumatologie Pédiatriques (UIHR), Hôpital Necker-Enfants Malades, 149, Rue de Sèvres, F-75015 Paris, France, or INSERM UMR1163, Laboratory Genome Dynamics in the Immune System, Imagine Institute, 24 boulevard du Montparnasse, 75015 Paris, France. Phone: 33.1.44.49.48.23; Email: despina.moshous@inserm.fr.

Authorship note: MBN and FA are co–first authors and contributed equally to this work. EM and C Boussard contributed equally to this work.

Find articles by Callebaut, I. in: JCI | PubMed | Google Scholar

1Université Paris Cité, Paris, France.

2Imagine Institute, Laboratory of Genome Dynamics in the Immune System, Equipe Labellisée Ligue Contre le Cancer, Ligue 2023, INSERM UMR 1163, Paris, France.

3General Pediatrics–Infectious Diseases and Internal Medicine, Hôpital Robert Debré, Assistance Publique-Hôpitaux de Paris (AP-HP) Nord, Paris, France.

4INSERM UMR S 1176, Laboratory for Hemostasis, Inflammation and Thrombosis (HITh), Université Paris-Saclay, Le Kremlin-Bicêtre, France.

5Laboratory Lymphocyte Activation and Susceptibility to EBV infection, INSERM UMR 1163, Imagine Institute, Paris, France.

6Pediatric Immunology, Hematology and Rheumatology, Necker-Enfants Malades University Hospital, AP-HP, Paris, France.

7Laboratory Immunogenetics of Pediatric Autoimmune Diseases, INSERM UMR 1163, Imagine Institute, Paris, France.

8Biotherapy Clinical Investigation Center, AP-HP, Paris, France.

9Laboratory Human Lymphohematopoiesis, INSERM UMR 1163, Imagine Institute, Paris, France.

10Sorbonne University, Muséum National d’Histoire Naturelle, UMR CNRS 7590, Institut de Minéralogie, de Physique des Matériaux et de Cosmochimie, IMPMC, Paris, France.

11Laboratoire d’Hémostase, Centre de Biologie Est, Hospices Civils de Lyon, Bron, France.

12Laboratory of Onco-Hematology, Necker-Enfants Malades University Hospital, AP-HP, Paris, France, and INSERM U1151, Institut Necker-Enfants Malades, Paris, France.

13Genomics Core Facility, Institut Imagine-Structure Fédérative de Recherche Necker, INSERM U1163 and INSERM US24/CNRS UAR3633, Paris Descartes Sorbonne Paris Cité University, Paris, France.

14Laboratory “Molecular basis of altered immune homeostasis,” INSERM UMR 1163, Imagine Institute, Paris, France.

15Study Center for Primary Immunodeficiencies, Necker-Enfants Malades University Hospital, AP-HP, Paris, France.

16Centre de Référence des Déficits Immunitaires Héréditaires (CEREDIH), Necker-Enfants Malades University Hospital, AP-HP, Paris, France.

17CHU de Caen Normandie, Onco-Immunohématologie Pédiatrique, Caen, France.

Address correspondence to: Despina Moshous, Unité d’Immunologie, Hématologie et Rhumatologie Pédiatriques (UIHR), Hôpital Necker-Enfants Malades, 149, Rue de Sèvres, F-75015 Paris, France, or INSERM UMR1163, Laboratory Genome Dynamics in the Immune System, Imagine Institute, 24 boulevard du Montparnasse, 75015 Paris, France. Phone: 33.1.44.49.48.23; Email: despina.moshous@inserm.fr.

Authorship note: MBN and FA are co–first authors and contributed equally to this work. EM and C Boussard contributed equally to this work.

Find articles by Kauskot, A. in: JCI | PubMed | Google Scholar |

1Université Paris Cité, Paris, France.

2Imagine Institute, Laboratory of Genome Dynamics in the Immune System, Equipe Labellisée Ligue Contre le Cancer, Ligue 2023, INSERM UMR 1163, Paris, France.

3General Pediatrics–Infectious Diseases and Internal Medicine, Hôpital Robert Debré, Assistance Publique-Hôpitaux de Paris (AP-HP) Nord, Paris, France.

4INSERM UMR S 1176, Laboratory for Hemostasis, Inflammation and Thrombosis (HITh), Université Paris-Saclay, Le Kremlin-Bicêtre, France.

5Laboratory Lymphocyte Activation and Susceptibility to EBV infection, INSERM UMR 1163, Imagine Institute, Paris, France.

6Pediatric Immunology, Hematology and Rheumatology, Necker-Enfants Malades University Hospital, AP-HP, Paris, France.

7Laboratory Immunogenetics of Pediatric Autoimmune Diseases, INSERM UMR 1163, Imagine Institute, Paris, France.

8Biotherapy Clinical Investigation Center, AP-HP, Paris, France.

9Laboratory Human Lymphohematopoiesis, INSERM UMR 1163, Imagine Institute, Paris, France.

10Sorbonne University, Muséum National d’Histoire Naturelle, UMR CNRS 7590, Institut de Minéralogie, de Physique des Matériaux et de Cosmochimie, IMPMC, Paris, France.

11Laboratoire d’Hémostase, Centre de Biologie Est, Hospices Civils de Lyon, Bron, France.

12Laboratory of Onco-Hematology, Necker-Enfants Malades University Hospital, AP-HP, Paris, France, and INSERM U1151, Institut Necker-Enfants Malades, Paris, France.

13Genomics Core Facility, Institut Imagine-Structure Fédérative de Recherche Necker, INSERM U1163 and INSERM US24/CNRS UAR3633, Paris Descartes Sorbonne Paris Cité University, Paris, France.

14Laboratory “Molecular basis of altered immune homeostasis,” INSERM UMR 1163, Imagine Institute, Paris, France.

15Study Center for Primary Immunodeficiencies, Necker-Enfants Malades University Hospital, AP-HP, Paris, France.

16Centre de Référence des Déficits Immunitaires Héréditaires (CEREDIH), Necker-Enfants Malades University Hospital, AP-HP, Paris, France.

17CHU de Caen Normandie, Onco-Immunohématologie Pédiatrique, Caen, France.

Address correspondence to: Despina Moshous, Unité d’Immunologie, Hématologie et Rhumatologie Pédiatriques (UIHR), Hôpital Necker-Enfants Malades, 149, Rue de Sèvres, F-75015 Paris, France, or INSERM UMR1163, Laboratory Genome Dynamics in the Immune System, Imagine Institute, 24 boulevard du Montparnasse, 75015 Paris, France. Phone: 33.1.44.49.48.23; Email: despina.moshous@inserm.fr.

Authorship note: MBN and FA are co–first authors and contributed equally to this work. EM and C Boussard contributed equally to this work.

Find articles by Repérant, C. in: JCI | PubMed | Google Scholar

1Université Paris Cité, Paris, France.

2Imagine Institute, Laboratory of Genome Dynamics in the Immune System, Equipe Labellisée Ligue Contre le Cancer, Ligue 2023, INSERM UMR 1163, Paris, France.

3General Pediatrics–Infectious Diseases and Internal Medicine, Hôpital Robert Debré, Assistance Publique-Hôpitaux de Paris (AP-HP) Nord, Paris, France.

4INSERM UMR S 1176, Laboratory for Hemostasis, Inflammation and Thrombosis (HITh), Université Paris-Saclay, Le Kremlin-Bicêtre, France.

5Laboratory Lymphocyte Activation and Susceptibility to EBV infection, INSERM UMR 1163, Imagine Institute, Paris, France.

6Pediatric Immunology, Hematology and Rheumatology, Necker-Enfants Malades University Hospital, AP-HP, Paris, France.

7Laboratory Immunogenetics of Pediatric Autoimmune Diseases, INSERM UMR 1163, Imagine Institute, Paris, France.

8Biotherapy Clinical Investigation Center, AP-HP, Paris, France.

9Laboratory Human Lymphohematopoiesis, INSERM UMR 1163, Imagine Institute, Paris, France.

10Sorbonne University, Muséum National d’Histoire Naturelle, UMR CNRS 7590, Institut de Minéralogie, de Physique des Matériaux et de Cosmochimie, IMPMC, Paris, France.

11Laboratoire d’Hémostase, Centre de Biologie Est, Hospices Civils de Lyon, Bron, France.

12Laboratory of Onco-Hematology, Necker-Enfants Malades University Hospital, AP-HP, Paris, France, and INSERM U1151, Institut Necker-Enfants Malades, Paris, France.

13Genomics Core Facility, Institut Imagine-Structure Fédérative de Recherche Necker, INSERM U1163 and INSERM US24/CNRS UAR3633, Paris Descartes Sorbonne Paris Cité University, Paris, France.

14Laboratory “Molecular basis of altered immune homeostasis,” INSERM UMR 1163, Imagine Institute, Paris, France.

15Study Center for Primary Immunodeficiencies, Necker-Enfants Malades University Hospital, AP-HP, Paris, France.

16Centre de Référence des Déficits Immunitaires Héréditaires (CEREDIH), Necker-Enfants Malades University Hospital, AP-HP, Paris, France.

17CHU de Caen Normandie, Onco-Immunohématologie Pédiatrique, Caen, France.

Address correspondence to: Despina Moshous, Unité d’Immunologie, Hématologie et Rhumatologie Pédiatriques (UIHR), Hôpital Necker-Enfants Malades, 149, Rue de Sèvres, F-75015 Paris, France, or INSERM UMR1163, Laboratory Genome Dynamics in the Immune System, Imagine Institute, 24 boulevard du Montparnasse, 75015 Paris, France. Phone: 33.1.44.49.48.23; Email: despina.moshous@inserm.fr.

Authorship note: MBN and FA are co–first authors and contributed equally to this work. EM and C Boussard contributed equally to this work.

Find articles by Feng, M. in: JCI | PubMed | Google Scholar

1Université Paris Cité, Paris, France.

2Imagine Institute, Laboratory of Genome Dynamics in the Immune System, Equipe Labellisée Ligue Contre le Cancer, Ligue 2023, INSERM UMR 1163, Paris, France.

3General Pediatrics–Infectious Diseases and Internal Medicine, Hôpital Robert Debré, Assistance Publique-Hôpitaux de Paris (AP-HP) Nord, Paris, France.

4INSERM UMR S 1176, Laboratory for Hemostasis, Inflammation and Thrombosis (HITh), Université Paris-Saclay, Le Kremlin-Bicêtre, France.

5Laboratory Lymphocyte Activation and Susceptibility to EBV infection, INSERM UMR 1163, Imagine Institute, Paris, France.

6Pediatric Immunology, Hematology and Rheumatology, Necker-Enfants Malades University Hospital, AP-HP, Paris, France.

7Laboratory Immunogenetics of Pediatric Autoimmune Diseases, INSERM UMR 1163, Imagine Institute, Paris, France.

8Biotherapy Clinical Investigation Center, AP-HP, Paris, France.

9Laboratory Human Lymphohematopoiesis, INSERM UMR 1163, Imagine Institute, Paris, France.

10Sorbonne University, Muséum National d’Histoire Naturelle, UMR CNRS 7590, Institut de Minéralogie, de Physique des Matériaux et de Cosmochimie, IMPMC, Paris, France.

11Laboratoire d’Hémostase, Centre de Biologie Est, Hospices Civils de Lyon, Bron, France.

12Laboratory of Onco-Hematology, Necker-Enfants Malades University Hospital, AP-HP, Paris, France, and INSERM U1151, Institut Necker-Enfants Malades, Paris, France.

13Genomics Core Facility, Institut Imagine-Structure Fédérative de Recherche Necker, INSERM U1163 and INSERM US24/CNRS UAR3633, Paris Descartes Sorbonne Paris Cité University, Paris, France.

14Laboratory “Molecular basis of altered immune homeostasis,” INSERM UMR 1163, Imagine Institute, Paris, France.

15Study Center for Primary Immunodeficiencies, Necker-Enfants Malades University Hospital, AP-HP, Paris, France.

16Centre de Référence des Déficits Immunitaires Héréditaires (CEREDIH), Necker-Enfants Malades University Hospital, AP-HP, Paris, France.

17CHU de Caen Normandie, Onco-Immunohématologie Pédiatrique, Caen, France.

Address correspondence to: Despina Moshous, Unité d’Immunologie, Hématologie et Rhumatologie Pédiatriques (UIHR), Hôpital Necker-Enfants Malades, 149, Rue de Sèvres, F-75015 Paris, France, or INSERM UMR1163, Laboratory Genome Dynamics in the Immune System, Imagine Institute, 24 boulevard du Montparnasse, 75015 Paris, France. Phone: 33.1.44.49.48.23; Email: despina.moshous@inserm.fr.

Authorship note: MBN and FA are co–first authors and contributed equally to this work. EM and C Boussard contributed equally to this work.

Find articles by Bordet, J. in: JCI | PubMed | Google Scholar

1Université Paris Cité, Paris, France.

2Imagine Institute, Laboratory of Genome Dynamics in the Immune System, Equipe Labellisée Ligue Contre le Cancer, Ligue 2023, INSERM UMR 1163, Paris, France.

3General Pediatrics–Infectious Diseases and Internal Medicine, Hôpital Robert Debré, Assistance Publique-Hôpitaux de Paris (AP-HP) Nord, Paris, France.

4INSERM UMR S 1176, Laboratory for Hemostasis, Inflammation and Thrombosis (HITh), Université Paris-Saclay, Le Kremlin-Bicêtre, France.

5Laboratory Lymphocyte Activation and Susceptibility to EBV infection, INSERM UMR 1163, Imagine Institute, Paris, France.

6Pediatric Immunology, Hematology and Rheumatology, Necker-Enfants Malades University Hospital, AP-HP, Paris, France.

7Laboratory Immunogenetics of Pediatric Autoimmune Diseases, INSERM UMR 1163, Imagine Institute, Paris, France.

8Biotherapy Clinical Investigation Center, AP-HP, Paris, France.

9Laboratory Human Lymphohematopoiesis, INSERM UMR 1163, Imagine Institute, Paris, France.

10Sorbonne University, Muséum National d’Histoire Naturelle, UMR CNRS 7590, Institut de Minéralogie, de Physique des Matériaux et de Cosmochimie, IMPMC, Paris, France.

11Laboratoire d’Hémostase, Centre de Biologie Est, Hospices Civils de Lyon, Bron, France.

12Laboratory of Onco-Hematology, Necker-Enfants Malades University Hospital, AP-HP, Paris, France, and INSERM U1151, Institut Necker-Enfants Malades, Paris, France.

13Genomics Core Facility, Institut Imagine-Structure Fédérative de Recherche Necker, INSERM U1163 and INSERM US24/CNRS UAR3633, Paris Descartes Sorbonne Paris Cité University, Paris, France.

14Laboratory “Molecular basis of altered immune homeostasis,” INSERM UMR 1163, Imagine Institute, Paris, France.

15Study Center for Primary Immunodeficiencies, Necker-Enfants Malades University Hospital, AP-HP, Paris, France.

16Centre de Référence des Déficits Immunitaires Héréditaires (CEREDIH), Necker-Enfants Malades University Hospital, AP-HP, Paris, France.

17CHU de Caen Normandie, Onco-Immunohématologie Pédiatrique, Caen, France.

Address correspondence to: Despina Moshous, Unité d’Immunologie, Hématologie et Rhumatologie Pédiatriques (UIHR), Hôpital Necker-Enfants Malades, 149, Rue de Sèvres, F-75015 Paris, France, or INSERM UMR1163, Laboratory Genome Dynamics in the Immune System, Imagine Institute, 24 boulevard du Montparnasse, 75015 Paris, France. Phone: 33.1.44.49.48.23; Email: despina.moshous@inserm.fr.

Authorship note: MBN and FA are co–first authors and contributed equally to this work. EM and C Boussard contributed equally to this work.

Find articles by Castelle, M. in: JCI | PubMed | Google Scholar

1Université Paris Cité, Paris, France.

2Imagine Institute, Laboratory of Genome Dynamics in the Immune System, Equipe Labellisée Ligue Contre le Cancer, Ligue 2023, INSERM UMR 1163, Paris, France.

3General Pediatrics–Infectious Diseases and Internal Medicine, Hôpital Robert Debré, Assistance Publique-Hôpitaux de Paris (AP-HP) Nord, Paris, France.

4INSERM UMR S 1176, Laboratory for Hemostasis, Inflammation and Thrombosis (HITh), Université Paris-Saclay, Le Kremlin-Bicêtre, France.

5Laboratory Lymphocyte Activation and Susceptibility to EBV infection, INSERM UMR 1163, Imagine Institute, Paris, France.

6Pediatric Immunology, Hematology and Rheumatology, Necker-Enfants Malades University Hospital, AP-HP, Paris, France.

7Laboratory Immunogenetics of Pediatric Autoimmune Diseases, INSERM UMR 1163, Imagine Institute, Paris, France.

8Biotherapy Clinical Investigation Center, AP-HP, Paris, France.

9Laboratory Human Lymphohematopoiesis, INSERM UMR 1163, Imagine Institute, Paris, France.

10Sorbonne University, Muséum National d’Histoire Naturelle, UMR CNRS 7590, Institut de Minéralogie, de Physique des Matériaux et de Cosmochimie, IMPMC, Paris, France.

11Laboratoire d’Hémostase, Centre de Biologie Est, Hospices Civils de Lyon, Bron, France.

12Laboratory of Onco-Hematology, Necker-Enfants Malades University Hospital, AP-HP, Paris, France, and INSERM U1151, Institut Necker-Enfants Malades, Paris, France.

13Genomics Core Facility, Institut Imagine-Structure Fédérative de Recherche Necker, INSERM U1163 and INSERM US24/CNRS UAR3633, Paris Descartes Sorbonne Paris Cité University, Paris, France.

14Laboratory “Molecular basis of altered immune homeostasis,” INSERM UMR 1163, Imagine Institute, Paris, France.

15Study Center for Primary Immunodeficiencies, Necker-Enfants Malades University Hospital, AP-HP, Paris, France.

16Centre de Référence des Déficits Immunitaires Héréditaires (CEREDIH), Necker-Enfants Malades University Hospital, AP-HP, Paris, France.

17CHU de Caen Normandie, Onco-Immunohématologie Pédiatrique, Caen, France.

Address correspondence to: Despina Moshous, Unité d’Immunologie, Hématologie et Rhumatologie Pédiatriques (UIHR), Hôpital Necker-Enfants Malades, 149, Rue de Sèvres, F-75015 Paris, France, or INSERM UMR1163, Laboratory Genome Dynamics in the Immune System, Imagine Institute, 24 boulevard du Montparnasse, 75015 Paris, France. Phone: 33.1.44.49.48.23; Email: despina.moshous@inserm.fr.

Authorship note: MBN and FA are co–first authors and contributed equally to this work. EM and C Boussard contributed equally to this work.

Find articles by Morelle, G. in: JCI | PubMed | Google Scholar |

1Université Paris Cité, Paris, France.

2Imagine Institute, Laboratory of Genome Dynamics in the Immune System, Equipe Labellisée Ligue Contre le Cancer, Ligue 2023, INSERM UMR 1163, Paris, France.

3General Pediatrics–Infectious Diseases and Internal Medicine, Hôpital Robert Debré, Assistance Publique-Hôpitaux de Paris (AP-HP) Nord, Paris, France.

4INSERM UMR S 1176, Laboratory for Hemostasis, Inflammation and Thrombosis (HITh), Université Paris-Saclay, Le Kremlin-Bicêtre, France.

5Laboratory Lymphocyte Activation and Susceptibility to EBV infection, INSERM UMR 1163, Imagine Institute, Paris, France.

6Pediatric Immunology, Hematology and Rheumatology, Necker-Enfants Malades University Hospital, AP-HP, Paris, France.

7Laboratory Immunogenetics of Pediatric Autoimmune Diseases, INSERM UMR 1163, Imagine Institute, Paris, France.

8Biotherapy Clinical Investigation Center, AP-HP, Paris, France.

9Laboratory Human Lymphohematopoiesis, INSERM UMR 1163, Imagine Institute, Paris, France.

10Sorbonne University, Muséum National d’Histoire Naturelle, UMR CNRS 7590, Institut de Minéralogie, de Physique des Matériaux et de Cosmochimie, IMPMC, Paris, France.

11Laboratoire d’Hémostase, Centre de Biologie Est, Hospices Civils de Lyon, Bron, France.

12Laboratory of Onco-Hematology, Necker-Enfants Malades University Hospital, AP-HP, Paris, France, and INSERM U1151, Institut Necker-Enfants Malades, Paris, France.

13Genomics Core Facility, Institut Imagine-Structure Fédérative de Recherche Necker, INSERM U1163 and INSERM US24/CNRS UAR3633, Paris Descartes Sorbonne Paris Cité University, Paris, France.

14Laboratory “Molecular basis of altered immune homeostasis,” INSERM UMR 1163, Imagine Institute, Paris, France.

15Study Center for Primary Immunodeficiencies, Necker-Enfants Malades University Hospital, AP-HP, Paris, France.

16Centre de Référence des Déficits Immunitaires Héréditaires (CEREDIH), Necker-Enfants Malades University Hospital, AP-HP, Paris, France.

17CHU de Caen Normandie, Onco-Immunohématologie Pédiatrique, Caen, France.

Address correspondence to: Despina Moshous, Unité d’Immunologie, Hématologie et Rhumatologie Pédiatriques (UIHR), Hôpital Necker-Enfants Malades, 149, Rue de Sèvres, F-75015 Paris, France, or INSERM UMR1163, Laboratory Genome Dynamics in the Immune System, Imagine Institute, 24 boulevard du Montparnasse, 75015 Paris, France. Phone: 33.1.44.49.48.23; Email: despina.moshous@inserm.fr.

Authorship note: MBN and FA are co–first authors and contributed equally to this work. EM and C Boussard contributed equally to this work.

Find articles by Brouzes, C. in: JCI | PubMed | Google Scholar

1Université Paris Cité, Paris, France.

2Imagine Institute, Laboratory of Genome Dynamics in the Immune System, Equipe Labellisée Ligue Contre le Cancer, Ligue 2023, INSERM UMR 1163, Paris, France.

3General Pediatrics–Infectious Diseases and Internal Medicine, Hôpital Robert Debré, Assistance Publique-Hôpitaux de Paris (AP-HP) Nord, Paris, France.

4INSERM UMR S 1176, Laboratory for Hemostasis, Inflammation and Thrombosis (HITh), Université Paris-Saclay, Le Kremlin-Bicêtre, France.

5Laboratory Lymphocyte Activation and Susceptibility to EBV infection, INSERM UMR 1163, Imagine Institute, Paris, France.

6Pediatric Immunology, Hematology and Rheumatology, Necker-Enfants Malades University Hospital, AP-HP, Paris, France.

7Laboratory Immunogenetics of Pediatric Autoimmune Diseases, INSERM UMR 1163, Imagine Institute, Paris, France.

8Biotherapy Clinical Investigation Center, AP-HP, Paris, France.

9Laboratory Human Lymphohematopoiesis, INSERM UMR 1163, Imagine Institute, Paris, France.

10Sorbonne University, Muséum National d’Histoire Naturelle, UMR CNRS 7590, Institut de Minéralogie, de Physique des Matériaux et de Cosmochimie, IMPMC, Paris, France.

11Laboratoire d’Hémostase, Centre de Biologie Est, Hospices Civils de Lyon, Bron, France.

12Laboratory of Onco-Hematology, Necker-Enfants Malades University Hospital, AP-HP, Paris, France, and INSERM U1151, Institut Necker-Enfants Malades, Paris, France.

13Genomics Core Facility, Institut Imagine-Structure Fédérative de Recherche Necker, INSERM U1163 and INSERM US24/CNRS UAR3633, Paris Descartes Sorbonne Paris Cité University, Paris, France.

14Laboratory “Molecular basis of altered immune homeostasis,” INSERM UMR 1163, Imagine Institute, Paris, France.

15Study Center for Primary Immunodeficiencies, Necker-Enfants Malades University Hospital, AP-HP, Paris, France.

16Centre de Référence des Déficits Immunitaires Héréditaires (CEREDIH), Necker-Enfants Malades University Hospital, AP-HP, Paris, France.

17CHU de Caen Normandie, Onco-Immunohématologie Pédiatrique, Caen, France.

Address correspondence to: Despina Moshous, Unité d’Immunologie, Hématologie et Rhumatologie Pédiatriques (UIHR), Hôpital Necker-Enfants Malades, 149, Rue de Sèvres, F-75015 Paris, France, or INSERM UMR1163, Laboratory Genome Dynamics in the Immune System, Imagine Institute, 24 boulevard du Montparnasse, 75015 Paris, France. Phone: 33.1.44.49.48.23; Email: despina.moshous@inserm.fr.

Authorship note: MBN and FA are co–first authors and contributed equally to this work. EM and C Boussard contributed equally to this work.

Find articles by Zarhrate, M. in: JCI | PubMed | Google Scholar

1Université Paris Cité, Paris, France.

2Imagine Institute, Laboratory of Genome Dynamics in the Immune System, Equipe Labellisée Ligue Contre le Cancer, Ligue 2023, INSERM UMR 1163, Paris, France.

3General Pediatrics–Infectious Diseases and Internal Medicine, Hôpital Robert Debré, Assistance Publique-Hôpitaux de Paris (AP-HP) Nord, Paris, France.

4INSERM UMR S 1176, Laboratory for Hemostasis, Inflammation and Thrombosis (HITh), Université Paris-Saclay, Le Kremlin-Bicêtre, France.

5Laboratory Lymphocyte Activation and Susceptibility to EBV infection, INSERM UMR 1163, Imagine Institute, Paris, France.

6Pediatric Immunology, Hematology and Rheumatology, Necker-Enfants Malades University Hospital, AP-HP, Paris, France.

7Laboratory Immunogenetics of Pediatric Autoimmune Diseases, INSERM UMR 1163, Imagine Institute, Paris, France.

8Biotherapy Clinical Investigation Center, AP-HP, Paris, France.

9Laboratory Human Lymphohematopoiesis, INSERM UMR 1163, Imagine Institute, Paris, France.

10Sorbonne University, Muséum National d’Histoire Naturelle, UMR CNRS 7590, Institut de Minéralogie, de Physique des Matériaux et de Cosmochimie, IMPMC, Paris, France.

11Laboratoire d’Hémostase, Centre de Biologie Est, Hospices Civils de Lyon, Bron, France.

12Laboratory of Onco-Hematology, Necker-Enfants Malades University Hospital, AP-HP, Paris, France, and INSERM U1151, Institut Necker-Enfants Malades, Paris, France.

13Genomics Core Facility, Institut Imagine-Structure Fédérative de Recherche Necker, INSERM U1163 and INSERM US24/CNRS UAR3633, Paris Descartes Sorbonne Paris Cité University, Paris, France.

14Laboratory “Molecular basis of altered immune homeostasis,” INSERM UMR 1163, Imagine Institute, Paris, France.

15Study Center for Primary Immunodeficiencies, Necker-Enfants Malades University Hospital, AP-HP, Paris, France.

16Centre de Référence des Déficits Immunitaires Héréditaires (CEREDIH), Necker-Enfants Malades University Hospital, AP-HP, Paris, France.

17CHU de Caen Normandie, Onco-Immunohématologie Pédiatrique, Caen, France.

Address correspondence to: Despina Moshous, Unité d’Immunologie, Hématologie et Rhumatologie Pédiatriques (UIHR), Hôpital Necker-Enfants Malades, 149, Rue de Sèvres, F-75015 Paris, France, or INSERM UMR1163, Laboratory Genome Dynamics in the Immune System, Imagine Institute, 24 boulevard du Montparnasse, 75015 Paris, France. Phone: 33.1.44.49.48.23; Email: despina.moshous@inserm.fr.

Authorship note: MBN and FA are co–first authors and contributed equally to this work. EM and C Boussard contributed equally to this work.

Find articles by Panikulam, P. in: JCI | PubMed | Google Scholar

1Université Paris Cité, Paris, France.

2Imagine Institute, Laboratory of Genome Dynamics in the Immune System, Equipe Labellisée Ligue Contre le Cancer, Ligue 2023, INSERM UMR 1163, Paris, France.

3General Pediatrics–Infectious Diseases and Internal Medicine, Hôpital Robert Debré, Assistance Publique-Hôpitaux de Paris (AP-HP) Nord, Paris, France.

4INSERM UMR S 1176, Laboratory for Hemostasis, Inflammation and Thrombosis (HITh), Université Paris-Saclay, Le Kremlin-Bicêtre, France.

5Laboratory Lymphocyte Activation and Susceptibility to EBV infection, INSERM UMR 1163, Imagine Institute, Paris, France.

6