EuroFlow-Based Flowcytometric Diagnostic Screening and Classification of Primary Immunodeficiencies of the Lymphoid System

DONGEN VAN, Jacques J. M., Mirjam BURG VAN DER, Tomas KALINA, Martin PEREZ-ANDRES, Ester MEJSTRIKOVA, Marcela VLKOVÁ, Eduardo LOPEZ-GRANADOS, Marjolein WENTINK, Anne-Kathrin KIENZLER, Jan PHILIPPE, Ana E. SOUSA, Menno C. ZELM VAN, Elena BLANCO and Alberto ORFAO. EuroFlow-Based Flowcytometric Diagnostic Screening and Classification of Primary Immunodeficiencies of the Lymphoid System. Frontiers in Immunology. LAUSANNE: FRONTIERS MEDIA SA, vol. 10, JUN 13 2019, p. 1-21. ISSN 1664-3224. doi:10.3389/fimmu.2019.01271. 2019.

Basic information

• Original name: EuroFlow-Based Flowcytometric Diagnostic Screening and Classification of Primary Immunodeficiencies of the Lymphoid System
• Authors: DONGEN VAN, Jacques J. M. (528 Netherlands, guarantor), Mirjam BURG VAN DER (528 Netherlands), Tomas KALINA (203 Czech Republic), Martin PEREZ-ANDRES (724 Spain), Ester MEJSTRIKOVA (203 Czech Republic), Marcela VLKOVÁ (203 Czech Republic, belonging to the institution), Eduardo LOPEZ-GRANADOS (724 Spain), Marjolein WENTINK (528 Netherlands), Anne-Kathrin KIENZLER (826 United Kingdom of Great Britain and Northern Ireland), Jan PHILIPPE (56 Belgium), Ana E. SOUSA (620 Portugal), Menno C. ZELM VAN (528 Netherlands), Elena BLANCO (724 Spain) and Alberto ORFAO (724 Spain).
• Edition: Frontiers in Immunology, LAUSANNE, FRONTIERS MEDIA SA, 2019, 1664-3224.

Other information

• Original language: English
• Type of outcome: Article in a journal
• Field of Study: 30102 Immunology
• Country of publisher: Switzerland
• Confidentiality degree: is not subject to a state or trade secret
• WWW: URL
• Impact factor: Impact factor: 5.085
• RIV identification code: RIV/00216224:14110/19:00112952
• Organization unit: Faculty of Medicine
• Doi: http://dx.doi.org/10.3389/fimmu.2019.01271
• UT WoS: 000471898900001
• Keywords in English: immunodeficiency; immunophenotyping; flow cytometry; diagnosis; classification; EuroFlow; standardization
• Tags: 14110114, rivok
• Tags: International impact, Reviewed

Abstract

Guidelines for screening for primary immunodeficiencies (PID) are well-defined and several consensus diagnostic strategies have been proposed. These consensus proposals have only partially been implemented due to lack of standardization in laboratory procedures, particularly in flow cytometry. The main objectives of the EuroFlow Consortium were to innovate and thoroughly standardize the flowcytometric techniques and strategies for reliable and reproducible diagnosis and classification of PID of the lymphoid system. The proposed EuroFlow antibody panels comprise one orientation tube and seven classification tubes and corresponding databases of normal and PID samples. The 8-color 12-antibody PID Orientation tube (PIDOT) aims at identification and enumeration of the main lymphocyte and leukocyte subsets; this includes naive pre-germinal center (GC) and antigen-experienced post-GC memory B-cells and plasmablasts. The seven additional 8(-12)-color tubes can be used according to the EuroFlow PID algorithm in parallel or subsequently to the PIDOT for more detailed analysis of B-cell and T-cell subsets to further classify PID of the lymphoid system. The Pre-GC, Post-GC, and immunoglobulin heavy chain (IgH)-isotype B-cell tubes aim at identification and enumeration of B-cell subsets for evaluation of B-cell maturation blocks and specific defects in IgH-subclass production. The severe combined immunodeficiency (SCID) tube and T-cell memory/effector subset tube aim at identification and enumeration of T-cell subsets for assessment of T-cell defects, such as SCID. In case of suspicion of antibody deficiency, PIDOT is preferably directly combined with the IgH isotype tube(s) and in case of SCID suspicion (e.g., in newborn screening programs) the PIDOT is preferably directly combined with the SCID T-cell tube. The proposed >= 8-color antibody panels and corresponding reference databases combined with the EuroFlow PID algorithm are designed to provide fast, sensitive and cost-effective flowcytometric diagnosis of PID of the lymphoid system, easily applicable in multicenter diagnostic settings world-wide.