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. Journal of allergy and clinical immunology. New York: Mosby-Elsevier, 2019, vol. 144, No 3, p. 809-824. ISSN 0091-6749. doi:10.1016/j.jaci.2019.02.017.
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Basic information
Original name Defects in memory B-cell and plasma cell subsets expressing different immunoglobulin-subclasses in patients with CVID and immunoglobulin subclass deficiencies
Authors BLANCO, Elena (724 Spain), Martin PEREZ-ANDRES (724 Spain), Sonia ARRIBA-MENDEZ (724 Spain), Cristina SERRANO (724 Spain), Ignacio CRIADO (724 Spain), Lucia DEL PINO-MOLINA (724 Spain), Susana SILVA (620 Portugal), Ignacio MADRUGA (724 Spain), Marina BAKARDJIEVA (203 Czech Republic), Catarina MARTINS (620 Portugal), Ana SERRA-CAETANO (620 Portugal), Alfonso ROMERO (724 Spain), Teresa CONTRERAS-SANFELICIANO (724 Spain), Carolien BONROY (56 Belgium), Francisco SALA (724 Spain), Alejandro MARTIN (724 Spain), Jose Maria BASTIDA (724 Spain), Felix LORENTE (724 Spain), Carlos PRIETO (724 Spain), Ignacio DAVILA (724 Spain), Miguel MARCOS (724 Spain), Tomas KALINA (203 Czech Republic), Marcela VLKOVÁ (203 Czech Republic, belonging to the institution), Zita CHOVANCOVÁ (203 Czech Republic, belonging to the institution), Ana Isabel CORDEIRO (620 Portugal), Jan PHILIPPE (56 Belgium), Filomeen HAERYNCK (56 Belgium), Eduardo LOPEZ-GRANADOS (724 Spain), Ana E. SOUSA (620 Portugal), Mirjam VAN DER BURG (528 Netherlands), Jacques J. M. VAN DONGEN (528 Netherlands) and Alberto ORFAO (724 Spain, guarantor).
Edition Journal of allergy and clinical immunology, New York, Mosby-Elsevier, 2019, 0091-6749.
Other information
Original language English
Type of outcome Article in a journal
Field of Study 30102 Immunology
Country of publisher United States of America
Confidentiality degree is not subject to a state or trade secret
WWW URL
Impact factor Impact factor: 10.228
RIV identification code RIV/00216224:14110/19:00112958
Organization unit Faculty of Medicine
Doi http://dx.doi.org/10.1016/j.jaci.2019.02.017
UT WoS 000485222300025
Keywords in English Immunodeficiency; primary antibody deficiency; selective IgA deficiency; common variable immunodeficiency; immunophenotyping; immunoglobulins; immunoglobulin subclasses; memory B cells; plasma cells; flow cytometry; diagnosis; classification
Tags 14110114, rivok
Tags International impact, Reviewed
Changed by Changed by: Mgr. Tereza Miškechová, učo 341652. Changed: 17/2/2020 10:35.
Abstract
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.
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