B-lymphocyte Subpopulations in Patients with Selective IgA Deficiency

Nechvátalová, Jana; Pikulova, Zdenka; Štikarovská, Dagmar; Pešák, Sáva; Vlková, Marcela

doi:10.1007/s10875-012-9655-6

Cited by 42 publications

(39 citation statements)

References 31 publications

(37 reference statements)

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“…Litzman et al [52] reported that there was no significant abnormality in the B-cell subsets of IgAD patients. However, further studies indicated decreased class-switched memory B cells in SIgAD patients [11, 12]. In this line, we indicated in one study that there are 2 subgroups of patients with IgAD (group I and II) based on switched memory B cells percentage [50].…”

Section: Pathogenesis Of Sigadmentioning

confidence: 99%

“…Few studies have evaluated the measurement of B-lymphocyte subsets in SIgAD patients [11, 12, 50-52]. Litzman et al [52] reported that there was no significant abnormality in the B-cell subsets of IgAD patients.…”

Section: Pathogenesis Of Sigadmentioning

confidence: 99%

“…Nechvatalova et al [11] evaluated similarities between CVID and IgAD based on the determination of B-lymphocyte subsets. They indicated a decrease in the frequency of switched memory cells, transitional cells as well as plasmablasts, and an increase in the CD21 low B cell subset compared to healthy individuals [53, 11].…”

Section: Pathogenesis Of Sigadmentioning

confidence: 99%

“…They indicated a decrease in the frequency of switched memory cells, transitional cells as well as plasmablasts, and an increase in the CD21 low B cell subset compared to healthy individuals [53, 11]. In this sense, Celiksoy et al [51] demonstrated high counts of naive B cells in patients with SIgAD similar to CVID patients.…”

Section: Pathogenesis Of Sigadmentioning

confidence: 99%

“…Based on the several efforts to clarify the etiology and pathogenesis of SIgAD, the major problem is the failure of B lymphocyte to produce IgA [11, 12]. However, other abnormalities such as increased lymphocytic apoptosis, cytokine network, signaling pathway by costimulatory molecules and also the presence of predisposing major histocompatibility complex (MHC) alleles might be involved in the pathogenesis of SIgAD [13-15].…”

Section: Introductionmentioning

confidence: 99%

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The Heterogeneous Pathogenesis of Selective Immunoglobulin A Deficiency

Bagheri¹,

Sanaei²,

Yazdani³

et al. 2019

Int Arch Allergy Immunol

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Selective immunoglobulin A deficiency (SIgAD) is the most prevalent type of primary immunodeficiency disorder. The phenotypic feature of SIgAD is related to a defect in B lymphocyte differentiation into plasma cell-producing immunoglobulin A (IgA). In this review, we summarize the recent advances in this regard. Genetic (including major histocompatibility complex [MHC] and non-MHC genes), immunologic (including B and T lymphocyte subsets abnormality), cytokines/chemokines and their related receptors, apoptosis and microbiota defects are reviewed. The mechanisms leading to SIgAD are most likely multifactorial and it can be speculated that several pathways controlling B cells functions or regulating epigenetic of the IGHA gene encoding constant region of IgA heavy chain and long-term survival of IgA switched memory B cells and plasma cells may be defective in different SIgAD patients.

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Section: Pathogenesis Of Sigadmentioning

confidence: 99%

Section: Pathogenesis Of Sigadmentioning

confidence: 99%

Section: Pathogenesis Of Sigadmentioning

confidence: 99%

Section: Pathogenesis Of Sigadmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

The Heterogeneous Pathogenesis of Selective Immunoglobulin A Deficiency

Bagheri¹,

Sanaei²,

Yazdani³

et al. 2019

Int Arch Allergy Immunol

View full text Add to dashboard Cite

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Defective formation of IgA memory B cells, Th1 and Th17 cells in symptomatic patients with selective IgA deficiency

et al. 2020

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Objective. Selective IgA deficiency (sIgAD) is the most common primary immunodeficiency in Western countries. Patients can suffer from recurrent infections and autoimmune diseases because of a largely unknown aetiology. To increase insights into the pathophysiology of the disease, we studied memory B and T cells and cytokine concentrations in peripheral blood. Methods. We analysed 30 sIgAD patients (12 children, 18 adults) through detailed phenotyping of peripheral B-cell, CD8 + T-cell and CD4 + Tcell subsets, sequence analysis of IGA and IGG transcripts, in vitro B-cell activation and blood cytokine measurements. Results. All patients had significantly decreased numbers of T-cell-dependent (TD; CD27 + ) and T-cell-independent (TI; CD27 À ) IgA memory B cells and increased CD21 low B-cell numbers. IgM + IgD À memory B cells were decreased in children and normal in adult patients. IGA and IGG transcripts contained normal SHM levels. In sIgAD children, IGA transcripts more frequently used IGA2 than controls (58.5% vs. 25.1%), but not in adult patients. B-cell activation after in vitro stimulation was normal. However, adult sIgAD patients exhibited increased blood levels of TGF-b1, BAFF and APRIL, whereas they had decreased Th1 and Th17 cell numbers. Conclusion. Impaired IgA memory formation in sIgAD patients is not due to a B-cell activation defect. Instead, decreased Th1 and Th17 cell numbers and high blood levels of BAFF, APRIL and TGF-b1 might reflect disturbed regulation of IgA responses in vivo.These insights into B-cell extrinsic immune defects suggest the need for a broader ª immunological focus on genomics and functional analyses to unravel the pathogenesis of sIgAD.

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