STUCHLÝ, Jan, Veronika KANDEROVÁ, Marcela VLKOVÁ, Ivana HEŘMANOVÁ, Lucie SLÁMOVÁ, Ondřej PELÁK, Eli TARALDSRUD, Dalibor JÍLEK, Pavlína KRALÍČKOVÁ, Borre FEVANG, Marie TRKOVÁ, Ondřej HRUŠÁK, Eva FROŇKOVÁ, Anna ŠEDIVÁ, Jiří LITZMAN and Tomáš KALINA. Common Variable Immunodeficiency patients with a phenotypic profile of immunosenescence present with thrombocytopenia. Scientific Reports. LONDON: NATURE PUBLISHING GROUP, 2017, vol. 7, No 39710, p. 1-12. ISSN 2045-2322. doi:10.1038/srep39710.
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Basic information
Original name Common Variable Immunodeficiency patients with a phenotypic profile of immunosenescence present with thrombocytopenia
Authors STUCHLÝ, Jan (203 Czech Republic), Veronika KANDEROVÁ (203 Czech Republic), Marcela VLKOVÁ (203 Czech Republic, belonging to the institution), Ivana HEŘMANOVÁ (203 Czech Republic), Lucie SLÁMOVÁ (203 Czech Republic), Ondřej PELÁK (203 Czech Republic), Eli TARALDSRUD (578 Norway), Dalibor JÍLEK (203 Czech Republic), Pavlína KRALÍČKOVÁ (203 Czech Republic), Borre FEVANG (578 Norway), Marie TRKOVÁ (203 Czech Republic), Ondřej HRUŠÁK (203 Czech Republic), Eva FROŇKOVÁ (203 Czech Republic), Anna ŠEDIVÁ (203 Czech Republic), Jiří LITZMAN (203 Czech Republic, guarantor, belonging to the institution) and Tomáš KALINA (203 Czech Republic).
Edition Scientific Reports, LONDON, NATURE PUBLISHING GROUP, 2017, 2045-2322.
Other information
Original language English
Type of outcome Article in a journal
Field of Study 30102 Immunology
Country of publisher United Kingdom of Great Britain and Northern Ireland
Confidentiality degree is not subject to a state or trade secret
Impact factor Impact factor: 4.122
RIV identification code RIV/00216224:14110/17:00096592
Organization unit Faculty of Medicine
Doi http://dx.doi.org/10.1038/srep39710
UT WoS 000391181500001
Keywords in English B-CELLS; CVID PATIENTS; T-CELLS; RHEUMATOID-ARTHRITIS; DIFFERENTIATION; DISEASE; CLASSIFICATION; MUTATIONS; ABNORMALITIES; DEFICIENCY
Tags EL OK
Tags International impact, Reviewed
Changed by Changed by: Soňa Böhmová, učo 232884. Changed: 20/3/2018 12:09.
Abstract
Common variable immunodeficiency (CVID) is a heterogeneous group of diseases. Our aim was to define sub-groups of CVID patients with similar phenotypes and clinical characteristics. Using eight-color flow cytometry, we analyzed both B-and T-cell phenotypes in a cohort of 88 CVID patients and 48 healthy donors. A hierarchical clustering of probability binning "bins" yielded a separate cluster of 22 CVID patients with an abnormal phenotype. We showed coordinated proportional changes in naive CD4+ T-cells (decreased), intermediate CD27-CD28+ CD4+ T-cells (increased) and CD21low B-cells (increased) that were stable for over three years. Moreover, the lymphocytes' immunophenotype in this patient cluster exhibited features of profound immunosenescence and chronic activation. Thrombocytopenia was only found in this cluster (36% of cases, manifested as Immune Thrombocytopenia (ITP) or Evans syndrome). Clinical complications more frequently found in these patients include lung fibrosis (in 59% of cases) and bronchiectasis (55%). The degree of severity of these symptoms corresponded to more deviation from normal levels with respect to CD21low B-cells, naive CD4+ and CD27-CD28+ over three years. Moreover, th-cells. Next-generation sequencing did not reveal any common genetic background. We delineate a subgroup of CVID patients with activated and immunosenescent immunophenotype of lymphocytes and distinct set of clinical complications without common genetic background.
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