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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 a 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, roč. 144, č. 3, s. 809-824. ISSN 0091-6749. doi:10.1016/j.jaci.2019.02.017.
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Základní údaje
Originální název Defects in memory B-cell and plasma cell subsets expressing different immunoglobulin-subclasses in patients with CVID and immunoglobulin subclass deficiencies
Autoři BLANCO, Elena (724 Španělsko), Martin PEREZ-ANDRES (724 Španělsko), Sonia ARRIBA-MENDEZ (724 Španělsko), Cristina SERRANO (724 Španělsko), Ignacio CRIADO (724 Španělsko), Lucia DEL PINO-MOLINA (724 Španělsko), Susana SILVA (620 Portugalsko), Ignacio MADRUGA (724 Španělsko), Marina BAKARDJIEVA (203 Česká republika), Catarina MARTINS (620 Portugalsko), Ana SERRA-CAETANO (620 Portugalsko), Alfonso ROMERO (724 Španělsko), Teresa CONTRERAS-SANFELICIANO (724 Španělsko), Carolien BONROY (56 Belgie), Francisco SALA (724 Španělsko), Alejandro MARTIN (724 Španělsko), Jose Maria BASTIDA (724 Španělsko), Felix LORENTE (724 Španělsko), Carlos PRIETO (724 Španělsko), Ignacio DAVILA (724 Španělsko), Miguel MARCOS (724 Španělsko), Tomas KALINA (203 Česká republika), Marcela VLKOVÁ (203 Česká republika, domácí), Zita CHOVANCOVÁ (203 Česká republika, domácí), Ana Isabel CORDEIRO (620 Portugalsko), Jan PHILIPPE (56 Belgie), Filomeen HAERYNCK (56 Belgie), Eduardo LOPEZ-GRANADOS (724 Španělsko), Ana E. SOUSA (620 Portugalsko), Mirjam VAN DER BURG (528 Nizozemsko), Jacques J. M. VAN DONGEN (528 Nizozemsko) a Alberto ORFAO (724 Španělsko, garant).
Vydání Journal of allergy and clinical immunology, New York, Mosby-Elsevier, 2019, 0091-6749.
Další údaje
Originální jazyk angličtina
Typ výsledku Článek v odborném periodiku
Obor 30102 Immunology
Stát vydavatele Spojené státy
Utajení není předmětem státního či obchodního tajemství
WWW URL
Impakt faktor Impact factor: 10.228
Kód RIV RIV/00216224:14110/19:00112958
Organizační jednotka Lékařská fakulta
Doi http://dx.doi.org/10.1016/j.jaci.2019.02.017
UT WoS 000485222300025
Klíčová slova anglicky Immunodeficiency; primary antibody deficiency; selective IgA deficiency; common variable immunodeficiency; immunophenotyping; immunoglobulins; immunoglobulin subclasses; memory B cells; plasma cells; flow cytometry; diagnosis; classification
Štítky 14110114, rivok
Příznaky Mezinárodní význam, Recenzováno
Změnil Změnila: Mgr. Tereza Miškechová, učo 341652. Změněno: 17. 2. 2020 10:35.
Anotace
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.
Zobrazeno: 18. 4. 2021 16:34