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@article{1623036,
author = {Blanco, Elena and PerezandAndres, Martin and ArribaandMendez, Sonia and Serrano, Cristina and Criado, Ignacio and Del PinoandMolina, Lucia and Silva, Susana and Madruga, Ignacio and Bakardjieva, Marina and Martins, Catarina and SerraandCaetano, Ana and Romero, Alfonso and ContrerasandSanfeliciano, Teresa and Bonroy, Carolien and Sala, Francisco and Martin, Alejandro and Bastida, Jose Maria and Lorente, Felix and Prieto, Carlos and Davila, Ignacio and Marcos, Miguel and Kalina, Tomas and Vlková, Marcela and Chovancová, Zita and Cordeiro, Ana Isabel and Philippe, Jan and Haerynck, Filomeen and LopezandGranados, Eduardo and Sousa, Ana E. and van der Burg, Mirjam and van Dongen, Jacques J. M. and Orfao, Alberto},
article_location = {New York},
article_number = {3},
doi = {http://dx.doi.org/10.1016/j.jaci.2019.02.017},
keywords = {Immunodeficiency; primary antibody deficiency; selective IgA deficiency; common variable immunodeficiency; immunophenotyping; immunoglobulins; immunoglobulin subclasses; memory B cells; plasma cells; flow cytometry; diagnosis; classification},
language = {eng},
issn = {0091-6749},
journal = {Journal of allergy and clinical immunology},
title = {Defects in memory B-cell and plasma cell subsets expressing different immunoglobulin-subclasses in patients with CVID and immunoglobulin subclass deficiencies},
url = {http://dx.doi.org/10.1016/j.jaci.2019.02.017},
volume = {144},
year = {2019}
}
TY - JOUR
ID - 1623036
AU - Blanco, Elena - Perez-Andres, Martin - Arriba-Mendez, Sonia - Serrano, Cristina - Criado, Ignacio - Del Pino-Molina, Lucia - Silva, Susana - Madruga, Ignacio - Bakardjieva, Marina - Martins, Catarina - Serra-Caetano, Ana - Romero, Alfonso - Contreras-Sanfeliciano, Teresa - Bonroy, Carolien - Sala, Francisco - Martin, Alejandro - Bastida, Jose Maria - Lorente, Felix - Prieto, Carlos - Davila, Ignacio - Marcos, Miguel - Kalina, Tomas - Vlková, Marcela - Chovancová, Zita - Cordeiro, Ana Isabel - Philippe, Jan - Haerynck, Filomeen - Lopez-Granados, Eduardo - Sousa, Ana E. - van der Burg, Mirjam - van Dongen, Jacques J. M. - Orfao, Alberto
PY - 2019
TI - Defects in memory B-cell and plasma cell subsets expressing different immunoglobulin-subclasses in patients with CVID and immunoglobulin subclass deficiencies
JF - Journal of allergy and clinical immunology
VL - 144
IS - 3
SP - 809-824
EP - 809-824
PB - Mosby-Elsevier
SN - 00916749
KW - Immunodeficiency
KW - primary antibody deficiency
KW - selective IgA deficiency
KW - common variable immunodeficiency
KW - immunophenotyping
KW - immunoglobulins
KW - immunoglobulin subclasses
KW - memory B cells
KW - plasma cells
KW - flow cytometry
KW - diagnosis
KW - classification
UR - http://dx.doi.org/10.1016/j.jaci.2019.02.017
L2 - http://dx.doi.org/10.1016/j.jaci.2019.02.017
N2 - Background: Predominantly antibody deficiencies (PADs) are the most prevalent primary immunodeficiencies, but their B-cell defects and underlying genetic alterations remain largely unknown. Objective: We investigated patients with PADs for the distribution of 41 blood B-cell and plasma cell (PC) subsets, including subsets defined by expression of distinct immunoglobulin heavy chain subclasses. Methods: Blood samples from 139 patients with PADs, 61 patients with common variable immunodeficiency (CVID), 68 patients with selective IgA deficiency (IgAdef), 10 patients with IgG subclass deficiency with IgA deficiency, and 223 age matched control subjects were studied by using flow cytometry with EuroFlow immunoglobulin isotype staining. Patients were classified according to their B-cell and PC immune profile, and the obtained patient clusters were correlated with clinical manifestations of PADs. Results: Decreased counts of blood PCs, memory B cells (MB Cs), or both expressing distinct IgA and IgG subclasses were identified in all patients with PADs. In patients with IgAdef, B-cell defects were mainly restricted to surface membrane (sm)IgA(+) PCs and MBCs, with 2 clear subgroups showing strongly decreased numbers of smIgA(+) PCs with mild versus severe smIgA(+) MBC defects and higher frequencies of nonrespiratory tract infections, autoimmunity, and affected family members. Patients with IgG subclass deficiency with IgA deficiency and those with CVID showed defects in both smIgA(+) and smIgG(+) MBCs and PCs. Reduced numbers of switched PCs were systematically found in patients with CVID (absent in 98%), with 6 different defective MBC (and clinical) profiles: (1) profound decrease in MBC numbers; (2) defective CD27(+) MBCs with almost normal IgG(3)(+) MBCs; (3) absence of switched MBCs; and (4) presence of both unswitched and switched MBCs without and; (5) with IgG(2)(+) MBCs; and (6) with IgA(1)(+) MBCs. Conclusion: Distinct PAD defective B-cell patterns were identified that are associated with unique clinical profiles.
ER -
BLANCO, Elena, Martin PEREZ-ANDRES, Sonia ARRIBA-MENDEZ, Cristina SERRANO, Ignacio CRIADO, Lucia DEL PINO-MOLINA, Susana SILVA, Ignacio MADRUGA, Marina BAKARDJIEVA, Catarina MARTINS, Ana SERRA-CAETANO, Alfonso ROMERO, Teresa CONTRERAS-SANFELICIANO, Carolien BONROY, Francisco SALA, Alejandro MARTIN, Jose Maria BASTIDA, Felix LORENTE, Carlos PRIETO, Ignacio DAVILA, Miguel MARCOS, Tomas KALINA, Marcela VLKOVÁ, Zita CHOVANCOVÁ, Ana Isabel CORDEIRO, Jan PHILIPPE, Filomeen HAERYNCK, Eduardo LOPEZ-GRANADOS, Ana E. SOUSA, Mirjam VAN DER BURG, Jacques J. M. VAN DONGEN and Alberto ORFAO. Defects in memory B-cell and plasma cell subsets expressing different immunoglobulin-subclasses in patients with CVID and immunoglobulin subclass deficiencies. \textit{Journal of allergy and clinical immunology}. New York: Mosby-Elsevier, vol.~144, No~3, p.~809-824. ISSN~0091-6749. doi:10.1016/j.jaci.2019.02.017. 2019.
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