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

J 2017

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

STUCHLÝ, Jan, Veronika KANDEROVÁ, Marcela VLKOVÁ, Ivana HEŘMANOVÁ, Lucie SLÁMOVÁ et. al.

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

Language

English

Type of outcome

Článek v odborném periodiku

Field of Study

30102 Immunology

Country of publisher

United Kingdom of Great Britain and Northern Ireland

Confidentiality degree

není předmětem státního či obchodního tajemství

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

Abstract

V originále

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.

Citovat

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. Available from: https://dx.doi.org/10.1038/srep39710.

@article{1379560,
   author = {Stuchlý, Jan and Kanderová, Veronika and Vlková, Marcela and Heřmanová, Ivana and Slámová, Lucie and Pelák, Ondřej and Taraldsrud, Eli and Jílek, Dalibor and Kralíčková, Pavlína and Fevang, Borre and Trková, Marie and Hrušák, Ondřej and Froňková, Eva and Šedivá, Anna and Litzman, Jiří and Kalina, Tomáš},
   article_location = {LONDON},
   article_number = {39710},
   doi = {http://dx.doi.org/10.1038/srep39710},
   keywords = {B-CELLS; CVID PATIENTS; T-CELLS; RHEUMATOID-ARTHRITIS; DIFFERENTIATION; DISEASE; CLASSIFICATION; MUTATIONS; ABNORMALITIES; DEFICIENCY},
   language = {eng},
   issn = {2045-2322},
   journal = {Scientific Reports},
   title = {Common Variable Immunodeficiency patients with a phenotypic profile of immunosenescence present with thrombocytopenia},
   volume = {7},
   year = {2017}
}

TY  - JOUR
ID  - 1379560
AU  - Stuchlý, Jan - Kanderová, Veronika - Vlková, Marcela - Heřmanová, Ivana - Slámová, Lucie - Pelák, Ondřej - Taraldsrud, Eli - Jílek, Dalibor - Kralíčková, Pavlína - Fevang, Borre - Trková, Marie - Hrušák, Ondřej - Froňková, Eva - Šedivá, Anna - Litzman, Jiří - Kalina, Tomáš
PY  - 2017
TI  - Common Variable Immunodeficiency patients with a phenotypic profile of immunosenescence present with thrombocytopenia
JF  - Scientific Reports
VL  - 7
IS  - 39710
SP  - 1-12
EP  - 1-12
PB  - NATURE PUBLISHING GROUP
SN  - 20452322
KW  - B-CELLS
KW  - CVID PATIENTS
KW  - T-CELLS
KW  - RHEUMATOID-ARTHRITIS
KW  - DIFFERENTIATION
KW  - DISEASE
KW  - CLASSIFICATION
KW  - MUTATIONS
KW  - ABNORMALITIES
KW  - DEFICIENCY
N2  - 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.
ER  -

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. \textit{Scientific Reports}. LONDON: NATURE PUBLISHING GROUP, 2017, vol.~7, No~39710, p.~1-12. ISSN~2045-2322. Available from: https://dx.doi.org/10.1038/srep39710.

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