Age dependency and mutual relations in T and B lymphocyte abnormalities in common variable immunodeficiency patients

Vlková, Marcela; Thon, Vojtěch; Šárfyová, Mária; Bláha, Luděk; Svobodník, Adam; Lokaj, J; Litzman, Jiří

doi:10.1111/j.1365-2249.2006.02999.x

Cited by 41 publications

(41 citation statements)

References 28 publications

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“…These abnormalities include an increase in HLA-DR, CD29, CD45RO, and CD95 (APO-1/Fas) and a decrease in CD27, CD45RA, and CD62L, especially in patients with increased CD21 low B-cell counts and decreased memory and regulatory B-cell counts [25,44]. CVID patients present with both low regulatory B cell counts and increased IFN-γ + TNF-α + CD4…”

Section: Activated Cd4 + T Cellsmentioning

confidence: 99%

“…Traditionally, the T-cell abnormalities that characterize CVID included a reduced T-cell count, decreased lymphocyte proliferation in response to mitogens and antigens, defective T-cell signaling, decline of regulatory T cell (Tregs) counts, uncontrolled T-cell polarization, elevated levels of T-cell activation markers, and abnormality in cytokine production secondary to gene polymorphisms [12][13][14][21][22][23][24][25][26].…”

Section: T-cell Abnormalities In Cvidmentioning

confidence: 99%

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T-Cell Abnormalities in Common Variable Immunodeficiency

Azizi

Rezaei

Kiaee

et al. 2016

J Investig Allergol Clin Immunol

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Common variable immunodeficiency (CVID) is the most common clinical primary immunodeficiency. It is characterized by a defect in B-cell differentiation to plasma and memory B cells. Moreover, numerous T-cell abnormalities have been reported and include decreased T-cell count and proliferative response, increased T-cell activation and apoptosis, and abnormalities in cytokine production. The aims of this review are to describe phenotypic and functional defects in T cells in CVID patients and to review the literature with respect to the effects of immunoglobulin replacement on the T-cell component in CVID patients. Key words: Common variable immunodeficiency. T cell. Helper T cells. Regulatory T cells. ResumenLa inmunodeficiencia común variable (CVID) es la inmunodeficiencia primaria más frecuente. Se caracteriza por un defecto en la diferenciación de linfocitos B hacia células plasmáticas y linfocitos B memoria. Se han descrito numerosas alteraciones en los linfocitos T de estos pacientes, tales como disminución en el número de linfocitos T y en sus respuestas proliferativas, aumento en la activación de células T y en la apoptosis, así como alteraciones en la producción de citokinas. El objetivo de esta revisión es describir las alteraciones funcionales y fenotípicas de los linfocitos T en los pacientes con CVID y revisar la bibliografía en relación a los efectos que la administración de inmunoglobulinas produce en los linfocitos T de los pacientes con CVID. Palabras clave: Inmunodeficiencia común variable. Células T. Células T helper. Células T reguladoras.

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Section: Activated Cd4 + T Cellsmentioning

confidence: 99%

Section: T-cell Abnormalities In Cvidmentioning

confidence: 99%

T-Cell Abnormalities in Common Variable Immunodeficiency

Azizi

Rezaei

Kiaee

et al. 2016

J Investig Allergol Clin Immunol

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“…Although various abnormalities in number and function of T cells (3)(4)(5)(6)(7), B cells (8)(9)(10)(11)(12)(13), and dendritic cells (14)(15)(16) have been described, the immunopathological mechanism leading to disturbed Ab production in CVID patients has not yet been defined.…”

Section: Ommon Variable Immunodeficiency (Cvid) Is a Clinically Hetmentioning

confidence: 99%

“…Previous studies have shown abnormalities in B cell subset composition -a diminution in memory B cells and increase in naive follicular (FO) B cells (5). Naive FO B cells IgM pos CD27 neg (17,18) constitute a major B cell subset in the peripheral blood of patients with CVID.…”

Section: Ommon Variable Immunodeficiency (Cvid) Is a Clinically Hetmentioning

confidence: 99%

Characterization of Lymphocyte Subsets in Patients with Common Variable Immunodeficiency Reveals Subsets of Naive Human B Cells Marked by CD24 Expression

Vlková

Froňková

Kanderová

et al. 2010

The Journal of Immunology

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Increased proportions of naive B cell subset and B cells defined as CD27negCD21negCD38neg are frequently found in patients with common variable immunodeficiency (CVID) syndrome. Current methods of polychromatic flow cytometry and PCR-based detection of κ deletion excision circles allow for fine definitions and replication history mapping of infrequent B cell subsets. We have analyzed B cells from 48 patients with CVID and 49 healthy controls to examine phenotype, frequency, and proliferation history of naive B cell subsets. Consistent with previous studies, we have described two groups of patients with normal (CVID-21norm) or increased (CVID-21lo) proportions of CD27negCD21negCD38neg B cells. Upon further analyses, we found two discrete subpopulations of this subset based on the expression of CD24. The B cell subsets showed a markedly increased proliferation in CVID-21lo patients as compared with healthy controls, suggesting developmental arrest rather than increased bone marrow output. Furthermore, when we analyzed CD21pos naive B cells, we found two different subpopulations based on IgM and CD24 expression. They correspond to follicular (FO) I and FO II cells previously described in mice. FO I subset is significantly underrepresented in CVID-21lo patients. A comparison of the replication history of naive B cell subsets in CVID patients and healthy controls implies refined naive B cell developmental scheme, in which human transitional B cells develop into FO II and FO I. We propose that the CD27negCD21negCD38neg B cells increased in some of the CVID patients originate from the two FO subsets after loss of CD21 expression.

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“…(2). Several research groups have found correlations between B-cell compartment abnormities and clinical presentations (1,3,4); however, the causal relationship between the defective B-cell function and the clinical picture is still elusive.…”

mentioning

confidence: 99%

Profiling of polychromatic flow cytometry data on B‐cells reveals patients' clusters in common variable immunodeficiency

et al. 2009

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The aim of this study was to find an objective computational approach for phenotype analysis of common variable immunodeficiency (CVID) patients that describes all differences in the six-color space and to form groups of patients using computational methods. CVID is a heterogeneous primary immunodeficiency disorder where molecular defect is recognized in \10% of the cases and is unknown in the majority of patients. The current CVID classification, EUROClass, is based on quantification of selected B-cell subsets. Using six-color polychromatic flow cytometry, we analyzed B-cell phenotypes in a cohort of 48 CVID patients and 49 healthy donors. We used a ''probability binning'' algorithm to create 1,024 bins (each bin is a six-color gate) that covered the cells' distribution within the entire B-cell compartment. A matrix file recording cellular content in all the bins was made. The hierarchical clustering of the individual samples was analyzed using a Pearson correlation of the bins' values. The Cut tree algorithm found 12 clusters. In six clusters, healthy individuals predominated; in one cluster, smB1CD21low (CVID patients by EUROClass) cells prevailed; in one cluster, smB2CD21norm cells prevailed; in one cluster, smB1CD21low cells prevailed; the remaining cluster was mixed. The overall reproducibility of probability binning clustering was confirmed by matching of replicates to the original cohort using the similarity matrix of the Pearson correlation, 15 replicates matched the same individual, three replicates matched a different individual within the same cluster, and three replicates matched to a different cluster. We were able to define B-cell subsets over-or under-represented in a particular cluster and display them back in the flow cytometry software. We describe a new analytical approach that enables a search in an objective computational environment for patient cohorts that are defined by similar B-cell profiles and thus contribute to the description of differences between CVID patient groups. ' 2009 International Society for Advancement of CytometryKey terms B-lymphocyte subsets; common variable immunodeficiency; flow cytometry; probability binning; B-lymphocyte profiling THE peripheral blood B-cell compartment is a dynamic complex of B-cell subsets that undergo maturational steps, antigen exposure, memory cell formation, memory maintenance, and effector functions (antibody secretion). Polychromatic flow cytometry offers an extraordinary tool to investigate such a complex set of cell types. It allows for the definition and proportional assessment of B-cell subsets and also subdivides into less well-characterized intermediates. However, the analysis of complex data obtained from the six-color space is difficult. The standard analysis strategy uses the creation of series of two-dimensional gates, which makes the resulting analysis subjective, and ''a priori'' unknown small subsets are difficult to discover. Analysis of

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Age dependency and mutual relations in T and B lymphocyte abnormalities in common variable immunodeficiency patients

Cited by 41 publications

References 28 publications

T-Cell Abnormalities in Common Variable Immunodeficiency

T-Cell Abnormalities in Common Variable Immunodeficiency

Characterization of Lymphocyte Subsets in Patients with Common Variable Immunodeficiency Reveals Subsets of Naive Human B Cells Marked by CD24 Expression

Profiling of polychromatic flow cytometry data on B‐cells reveals patients' clusters in common variable immunodeficiency

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