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Defects in memory B-cell and plasma cell subsets expressing different immunoglobulin-subclasses in patients with CVID and immunoglobulin subclass deficiencies

2019-09Artigo científico Acesso aberto
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dc.contributor.authorBlanco, Elena
dc.contributor.authorPérez-Andrés, Martín
dc.contributor.authorArriba-Méndez, Sonia
dc.contributor.authorSerrano, Cristina
dc.contributor.authorCriado, Ignacio
dc.contributor.authorDel Pino-Molina, Lucía
dc.contributor.authorSilva, Susana
dc.contributor.authorMadruga, Ignacio
dc.contributor.authorBakardjieva, Marina
dc.contributor.authorMartins, Catarina
dc.contributor.authorSerra-Caetano, Ana
dc.contributor.authorRomero, Alfonso
dc.contributor.authorContreras-Sanfeliciano, Teresa
dc.contributor.authorBonroy, Carolien
dc.contributor.authorSala, Francisco
dc.contributor.authorMartín, Alejandro
dc.contributor.authorBastida, José María
dc.contributor.authorLorente, Félix
dc.contributor.authorPrieto, Carlos
dc.contributor.authorDávila, Ignacio
dc.contributor.authorMarcos, Miguel
dc.contributor.authorKalina, Tomas
dc.contributor.authorVlkova, Marcela
dc.contributor.authorChovancova, Zita
dc.contributor.authorCordeiro, Ana Isabel
dc.contributor.authorPhilippé, Jan
dc.contributor.authorHaerynck, Filomeen
dc.contributor.authorLópez-Granados, Eduardo
dc.contributor.authorSousa, Ana E.
dc.contributor.authorvan der Burg, Mirjam
dc.contributor.authorvan Dongen, Jacques J.M.
dc.contributor.authorOrfao, Alberto
dc.contributor.institutionNOVA Medical School|Faculdade de Ciências Médicas (NMS|FCM)
dc.contributor.pblMOSBY-ELSEVIER
dc.date.accessioned2023-01-18T22:12:22Z
dc.date.available2023-01-18T22:12:22Z
dc.date.issued2019-09
dc.descriptionFunding: E.B. was supported by a grant from the Junta de Castilla y Leon (Fondo Social Europeo, ORDEN EDU/346/2013, Valladolid, Spain). This work was supported by the CB16/ 12/00400 and CB/16/12/00233 grants (CIBERONC, Instituto de Salud Carlos III, Ministerio de Economıa y Competitividad, Madrid, Spain, and FONDOS FEDER), the FIS PI12/00905-FEDER grant (Fondo de Investigacion Sanitaria of Instituto de Salud Carlos III, Madrid, Spain) and a grant from Fundacion Mutua Madrile~na (Madrid, Spain). The coordination and innovation processes of this study were supported by the EuroFlow Consortium.
dc.description.abstractBackground: 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 (MBCs), 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.en
dc.description.versionpublishersversion
dc.description.versionpublished
dc.format.extent2719002
dc.identifier.doi10.1016/j.jaci.2019.02.017
dc.identifier.issn0091-6749
dc.identifier.otherPURE: 12746797
dc.identifier.otherPURE UUID: da8f246e-1747-423b-b519-3a29bfb0f8da
dc.identifier.otherScopus: 85063670071
dc.identifier.otherORCID: /0000-0003-0353-0421/work/64040769
dc.identifier.otherPubMed: 30826363
dc.identifier.otherWOS: 000485222300025
dc.identifier.urihttp://hdl.handle.net/10362/147794
dc.identifier.urlhttps://www.scopus.com/pages/publications/85063670071
dc.language.isoeng
dc.peerreviewedyes
dc.subjectclassification
dc.subjectcommon variable immunodeficiency
dc.subjectdiagnosis
dc.subjectflow cytometry
dc.subjectImmunodeficiency
dc.subjectimmunoglobulin subclasses
dc.subjectimmunoglobulins
dc.subjectimmunophenotyping
dc.subjectmemory B cells
dc.subjectplasma cells
dc.subjectprimary antibody deficiency
dc.subjectselective IgA deficiency
dc.subjectImmunology and Allergy
dc.subjectImmunology
dc.titleDefects in memory B-cell and plasma cell subsets expressing different immunoglobulin-subclasses in patients with CVID and immunoglobulin subclass deficienciesen
dc.typejournal article
degois.publication.firstPage809
degois.publication.issue3
degois.publication.lastPage824
degois.publication.titleJournal Of Allergy And Clinical Immunology
degois.publication.volume144
dspace.entity.typePublication
rcaap.rightsopenAccess

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