Common Variable Immunodeficiency patients with a phenotypic profile of immunosenescence present with thrombocytopenia

Result code in IS VaVaI

<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00216208%3A11130%2F17%3A10360722" target="_blank" >RIV/00216208:11130/17:10360722 - isvavai.cz</a>

Alternative codes found

RIV/00216224:14110/17:00096592 RIV/00179906:_____/17:10360722 RIV/00159816:_____/17:00067203 RIV/00064203:_____/17:10360722

Result on the web

<a href="https://www.nature.com/articles/srep39710" target="_blank" >https://www.nature.com/articles/srep39710</a>

DOI - Digital Object Identifier

<a href="http://dx.doi.org/10.1038/srep39710" target="_blank" >10.1038/srep39710</a>

Result language

angličtina

Original language name

Common Variable Immunodeficiency patients with a phenotypic profile of immunosenescence present with thrombocytopenia

Original language description

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 &quot;bins&quot; 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&apos; 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.

Czech name

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Czech description

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Type

J_imp - Article in a specialist periodical, which is included in the Web of Science database

CEP classification

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OECD FORD branch

30204 - Oncology

Project

Result was created during the realization of more than one project. More information in the Projects tab.

Continuities

P - Projekt vyzkumu a vyvoje financovany z verejnych zdroju (s odkazem do CEP)

Publication year

2017

Confidentiality

S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Name of the periodical

Scientific Reports

ISSN

2045-2322

e-ISSN

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Volume of the periodical

7

Issue of the periodical within the volume

January

Country of publishing house

GB - UNITED KINGDOM

Number of pages

12

Pages from-to

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UT code for WoS article

000391181500001

EID of the result in the Scopus database

2-s2.0-85008608328