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

Stuchlý, Jan; Kanderová, Veronika; Vlková, Marcela; Heřmanová, Ivana; Slámová, Lucie; Pelák, Ondřej; Taraldsrud, Eli; Jílek, Dalibor; Králíčková, Pavlína; Fevang, Børre; Trková, Marie; Hrušák, Ondřej; Froňková, Eva; Šedivá, Anna; Litzman, Jiří; Kalina, Tomáš

doi:10.1038/srep39710

Cited by 26 publications

(18 citation statements)

References 52 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Not surprisingly, manual analysis influences the end result to a great extent. To complicate the matter, particular diseases present with high proportions of subsets that are very rare in healthy (4,5) necessitating disease specific benchmarking. 1A), but would yield larger discrepancy in CD8+ T-cells (Fig.…”

Section: Why At What Level and To What Extent We Need Reproducibilitymentioning

confidence: 99%

“…1B), stressing the need for subset specific benchmarking. To complicate the matter, particular diseases present with high proportions of subsets that are very rare in healthy (4,5) necessitating disease specific benchmarking. Definitions that influence staining patterns may impact the ability to clearly define and/or resolve particular subsets, which in turn will impact enumeration.…”

Section: Why At What Level and To What Extent We Need Reproducibilitymentioning

confidence: 99%

See 1 more Smart Citation

Reproducibility of Flow Cytometry Through Standardization: Opportunities and Challenges

Kalina

2019

Cytometry Pt A

View full text Add to dashboard Cite

There is an agreement in the field that interlaboratory reproducibility of flow cytometry measurements as well as the whole studies might be improved by a consensual use of methodological approach. Typically, a consensus is made on a crucial markers needed in the immunostaining panel, sometimes on the particular fluorochrome conjugates and rarely on a complete set of methods for sample preparation. The term "standardization" is used to describe the complete set of methodical steps, while "harmonization" is used for partial agreement on the method. Standardization can provide a platform for improved reproducibility of cytometry results over prolonged periods of time, across different sites and across different instruments. For the purpose of structured discussion, several desired aims are described: common interpretation of the immunophenotype definition of a target subset, accurate quantification, reproducible pattern of a multicolor immunophenotype, and reproducible intensity of all measured parameters. An overview of how standardization was approached by several large consortia is provided: EuroFlow, The ONE Study, Human Immunology Project Consortium (HIPC), and several other groups. Their particular aims and the tools adopted to reach those aims are noted. How those standardization efforts were adopted in the field and how the resulting outcome was evaluated is reviewed. Multiple challenges in the instrument hardware design, instrument setup tools, reagent design, and quality features need to be addressed to achieve optimal standardization. Furthermore, the aims of different studies vary, and thus, the reasonable requirements for standardization differ. A framework of reference for the reasonable outcomes of different approaches is offered. Finally, it is argued that complete standardization is important not only for the reproducibility of measurements but also for education, for quality assessment and for algorithmic data analysis. The different standardized approaches can and in fact should serve as benchmarking reference tools for the development of future flow cytometry studies.

show abstract

Section: Why At What Level and To What Extent We Need Reproducibilitymentioning

confidence: 99%

Section: Why At What Level and To What Extent We Need Reproducibilitymentioning

confidence: 99%

Reproducibility of Flow Cytometry Through Standardization: Opportunities and Challenges

Kalina

2019

Cytometry Pt A

View full text Add to dashboard Cite

show abstract

“…The pathogenic mechanism of rheumatologic diseases in CVID is not yet recognized. However, increased autoreactive B cell such as CD21 low B cell, autoantibodies, reduced number of regulatory T cells (Tregs) and abnormalities in cytokine production may result in autoimmune conditions and rheumatologic diseases . Moreover, decreased IL‐10‐producing regulatory B cells (which play an important role in the development of Tregs and peripheral tolerance) have been reported in patients with CVID.…”

Section: Introductionmentioning

confidence: 99%

Rheumatologic complications in a cohort of 227 patients with common variable immunodeficiency

et al. 2018

View full text Add to dashboard Cite

Common variable immunodeficiency (CVID) is the most prevalent symptomatic type of human primary immunodeficiency diseases (PID). Clinically, CVID is characterized by increased susceptibility to infections and a wide variety of autoimmune and rheumatologic disorders. All patients with CVID registered in Iranian PID Registry (IPIDR) were enrolled in this retrospective cohort study. We investigated the frequency of rheumatologic diseases and its association with immunological and clinical phenotypes in patients with CVID. A total of 227 patients with CVID were enrolled in this study. The prevalence of rheumatologic disorders was 10.1% with a higher frequency in women than men. Most common rheumatologic manifestations were juvenile idiopathic arthritis (JIA) and adult rheumatoid arthritis (RA) followed by juvenile spondyloarthritis (JSpA) and undifferentiated inflammatory arthritis (UIA). Septic arthritis in patients with CVID with a history of RA and JIA was higher than patients without rheumatologic complication. Patients with CVID with a history of autoimmunity (both rheumatologic and non-rheumatologic autoimmunity) had lower regulatory T cells counts in comparison with patients without autoimmune disorders. There was an association between defect in specific antibody responses and negative serologic test results in patients with rheumatologic manifestations. JIA, RA, JSpA and UIA are the most frequent rheumatologic disorders in patients with CVID. Due to antibody deficiency, serologic tests may be negative in these patients. Therefore, these conditions pose significant diagnostic and therapeutic challenges for immunologists and rheumatologists in charge of the care for these patients.

show abstract

“…CVID is a heterogeneous disease, which is associated with recurrent and severe infections and characterized by deficiencies in antibody production. Previous studies have shown that there are subgroups of patients with CVID with respect to their genotype, B cell or T cell profiles and clinical phenotypes [10][11][12][13][14][15][16]. Most CVID patients receive immunoglobulin (Ig)G substitution, which has a significant impact on health-related quality of life [8,9].…”

Section: Introductionmentioning

confidence: 99%

“…Most CVID patients receive immunoglobulin (Ig)G substitution, which has a significant impact on health-related quality of life [8,9]. Previous studies have shown that there are subgroups of patients with CVID with respect to their genotype, B cell or T cell profiles and clinical phenotypes [10][11][12][13][14][15][16]. However, very few studies analyzing the heterogeneity of CVID go beyond biomedical perspectives and focus on the psychosocial aspects of the disease including perceived health.…”

Section: Introductionmentioning

confidence: 99%

Perceived health of patients with common variable immunodeficiency – a cluster analysis

Bayrhuber

Tinsel

Goldacker

et al. 2019

Clinical and Experimental Immunology

View full text Add to dashboard Cite

Common variable immunodeficiency (CVID) is a complex disease with various influences on perceived health, which correlate with different outcomes, including new morbidity and mortality. Our hypothesis was that CVID patients fall into distinct clusters of perceived health which can inform care. Ward hierarchical cluster analysis and K-means cluster analysis were performed on data of 209 CVID patients to identify subgroups regarding their self-reported physical and mental health status, assessed by the physical (PCS) and mental component scores (MCS) of the Short Form-12 (SF-12). Four clusters of CVID-patients were identified. Cluster 1 was the largest cluster, characterized by a relatively high physical and mental health status (44·0%). In contrast, cluster 2 (21·1%) included patients with low physical and mental health status. Clusters 3 and 4 were mixed groups with high mental and low physical health (15·8%) and vice versa (19·1%). Significant differences between the clusters were found for patient-reported outcomes such as work ability and health literacy, but not for CVID-associated complications such as enteropathy, interstitial lung disease, granulomatosis, lymphadenopathy and autoimmune cytopenia or laboratory parameters such as immunoglobulin levels or B cell-based classification. The results suggest different subgroups of CVID patients with contrasting individual needs which, surprisingly, did not differ in clinical or laboratory characteristics. The main finding of this study is that patients with CVID fall into four distinct clusters according to perceived health, which are largely independent of CVID complications.

show abstract

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

Cited by 26 publications

References 52 publications

Reproducibility of Flow Cytometry Through Standardization: Opportunities and Challenges

Reproducibility of Flow Cytometry Through Standardization: Opportunities and Challenges

Rheumatologic complications in a cohort of 227 patients with common variable immunodeficiency

Perceived health of patients with common variable immunodeficiency – a cluster analysis

Contact Info

Product

Resources

About