Dissection of the Pre-Germinal Center B-Cell Maturation Pathway in Common Variable Immunodeficiency Based on Standardized Flow Cytometric EuroFlow Tools.


Journal

Frontiers in immunology
ISSN: 1664-3224
Titre abrégé: Front Immunol
Pays: Switzerland
ID NLM: 101560960

Informations de publication

Date de publication:
2020
Historique:
received: 08 09 2020
accepted: 29 12 2020
entrez: 8 3 2021
pubmed: 9 3 2021
medline: 22 6 2021
Statut: epublish

Résumé

Common Variable Immunodeficiency (CVID) is characterized by defective antibody production and hypogammaglobulinemia. Flow cytometry immunophenotyping of blood lymphocytes has become of great relevance for the diagnosis and classification of CVID, due to an impaired differentiation of mature post-germinal-center (GC) class-switched memory B-cells (MBC) and severely decreased plasmablast/plasma cell (Pb) counts. Here, we investigated in detail the pre-GC B-cell maturation compartment in blood of CVID patients. In this collaborative multicentric study the EuroFlow PID 8-color The Our results show that maturation of pre-GC B-cells in blood of CVID is systematically altered with up to four distinctly altered maturation profiles. Further studies, are necessary to better understand the impact of such alterations on the post-GC defects and the clinical heterogeneity of CVID.

Identifiants

pubmed: 33679693
doi: 10.3389/fimmu.2020.603972
pmc: PMC7925888
doi:

Substances chimiques

Antigens, CD 0

Types de publication

Journal Article Multicenter Study Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

603972

Informations de copyright

Copyright © 2021 del Pino-Molina, López-Granados, Lecrevisse, Torres Canizales, Pérez-Andrés, Blanco, Wentink, Bonroy, Nechvatalova, Milota, Kienzler, Philippé, Sousa, van der Burg, Kalina, van Dongen and Orfao.

Déclaration de conflit d'intérêts

JD, MB, TK, MP-A, EL-G, A-KK, EB, and AO each report being one of the inventors on the EuroFlow-owned patent PCT/NL 2015/050762 (Diagnosis of primary immunodeficiencies), which is licensed to Cytognos, a company that pays royalties to the EuroFlow Consortium.

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Auteurs

Lucía Del Pino-Molina (L)

Clinical Immunology Department, La Paz University Hospital and Lymphocyte Pathophysiology in Immunodeficiencies Group, La Paz Institute for Health Research (IdiPAZ) and Center for Biomedical Network Research on Rare Diseases (CIBERER U767), Madrid, Spain.

Eduardo López-Granados (E)

Clinical Immunology Department, La Paz University Hospital and Lymphocyte Pathophysiology in Immunodeficiencies Group, La Paz Institute for Health Research (IdiPAZ) and Center for Biomedical Network Research on Rare Diseases (CIBERER U767), Madrid, Spain.

Quentin Lecrevisse (Q)

Clinical and Translation Research Program, Cancer Research Centre (IBMCC, USAL-CSIC), Department of Medicine, Cytometry Service (NUCLEUS), University of Salamanca (USAL), Institute of Biomedical Research of Salamanca (IBSAL), Salamanca, Spain.
Biomedical Research Networking Centre Consortium of Oncology (CIBERONC) Instituto de salud Carlos III, Madrid, Spain.

Juan Torres Canizales (J)

Clinical Immunology Department, La Paz University Hospital and Lymphocyte Pathophysiology in Immunodeficiencies Group, La Paz Institute for Health Research (IdiPAZ) and Center for Biomedical Network Research on Rare Diseases (CIBERER U767), Madrid, Spain.

Martín Pérez-Andrés (M)

Clinical and Translation Research Program, Cancer Research Centre (IBMCC, USAL-CSIC), Department of Medicine, Cytometry Service (NUCLEUS), University of Salamanca (USAL), Institute of Biomedical Research of Salamanca (IBSAL), Salamanca, Spain.
Biomedical Research Networking Centre Consortium of Oncology (CIBERONC) Instituto de salud Carlos III, Madrid, Spain.

Elena Blanco (E)

Clinical and Translation Research Program, Cancer Research Centre (IBMCC, USAL-CSIC), Department of Medicine, Cytometry Service (NUCLEUS), University of Salamanca (USAL), Institute of Biomedical Research of Salamanca (IBSAL), Salamanca, Spain.
Biomedical Research Networking Centre Consortium of Oncology (CIBERONC) Instituto de salud Carlos III, Madrid, Spain.

Marjolein Wentink (M)

Department of Immunology, Erasmus University Medical Center (Erasmus MC), Rotterdam, Netherlands.

Carolien Bonroy (C)

Department of Laboratory Medicine, University Hospital Ghent, Ghent, Belgium.

Jana Nechvatalova (J)

Department of Allergology and Clinical Immunology, Faculty of Medicine, Masaryk University and St Anne's University Hospital in Brno, Brno, Czechia.

Tomas Milota (T)

Department of Immunology, Second Faculty of Medicine, Charles University and Motol University Hospital, Prague, Czechia.

Anne-Kathrin Kienzler (AK)

Nuffield Department of Medicine, Experimental Medicine Division, University of Oxford, Oxford, United Kingdom.

Jan Philippé (J)

Department of Laboratory Medicine, University Hospital Ghent, Ghent, Belgium.

Ana E Sousa (AE)

Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, Lisboa, Portugal.

Mirjam van der Burg (M)

Department of Pediatrics, Laboratory for Immunology, Leiden University Medical Center, Leiden, Netherlands.

Tomas Kalina (T)

CLIP - Childhood Leukemia Investigation Prague, Department of Pediatric Hematology and Oncology, 2nd Faculty of Medicine, Charles University and University Hospital Motol, Prague, Czechia.

Jacques J M van Dongen (JJM)

Department of Immunohematology and Blood Transfusion, Leiden University Medical Center (LUMC), Leiden, Netherlands.

Alberto Orfao (A)

Clinical and Translation Research Program, Cancer Research Centre (IBMCC, USAL-CSIC), Department of Medicine, Cytometry Service (NUCLEUS), University of Salamanca (USAL), Institute of Biomedical Research of Salamanca (IBSAL), Salamanca, Spain.
Biomedical Research Networking Centre Consortium of Oncology (CIBERONC) Instituto de salud Carlos III, Madrid, Spain.

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