scholarly journals Chronic meningococcaemia and immunoglobulin A deficiency

2010 ◽  
Vol 59 (11) ◽  
pp. 1375-1378 ◽  
Author(s):  
Arnaud Theulin ◽  
Murielle Rondeau-Lutz ◽  
Cornelia Kuhnert ◽  
Julien Boileau ◽  
Jean-Christophe Weber
Keyword(s):  
B Cells ◽  
Neisseria Meningitidis ◽  
Common Variable Immunodeficiency ◽  
Clinical Presentation ◽  
Immunoglobulin A ◽  
Iga Deficiency ◽  
Memory B Cells ◽  
Igm Deficiency ◽  
Common Variable

Chronic meningococcaemia is an unusual clinical presentation of Neisseria meningitidis infection. We describe the case of a patient, who presented with total IgA deficiency and partial IgM deficiency with a low switched memory B cells count, suggestive of a borderline form of common variable immunodeficiency (CVID). The role of IgA in the protection against Neisseria meningitidis, and the link between IgA deficiency and CVID are discussed.

Reexamining the role of TACI coding variants in common variable immunodeficiency and selective IgA deficiency

2007 ◽  
Vol 39 (4) ◽  
pp. 430-431 ◽  
Author(s):  
Emanuela Castigli ◽  
Stephen Wilson ◽  
Lilit Garibyan ◽  
Rima Rachid ◽  
Francisco Bonilla ◽  
...  
Keyword(s):  
Common Variable Immunodeficiency ◽  
Iga Deficiency ◽  
Selective Iga Deficiency ◽  
Coding Variants ◽  
Common Variable

scholarly journals Patients with Common Variable Immunodeficiency Complicated by Autoimmune Phenomena Have Lymphopenia and Reduced Treg, Th17, and NK Cells

2021 ◽  
Vol 10 (15) ◽  
pp. 3356
Author(s):  
Ewa Więsik-Szewczyk ◽  
Elżbieta Rutkowska ◽  
Iwona Kwiecień ◽  
Marcelina Korzeniowska ◽  
Dariusz Sołdacki ◽  
...  
Keyword(s):  
B Cells ◽  
Nk Cells ◽  
Common Variable Immunodeficiency ◽  
Lupus Erythematosus ◽  
Memory B Cells ◽  
T Cell Subsets ◽  
Primary Immune Deficiency ◽  
Immunological Markers ◽  
Autoimmune Phenomena ◽  
Common Variable

Most patients with primary immune deficiency suffer from recurrent infections; however, paradoxical autoimmune phenomena can also manifest. The aim of this study was to identify immunological markers of autoimmune phenomena associated with common variable immunodeficiency (CVID). The study included 33 adults with CVID divided into two groups: (1) those with noninfectious autoimmune complications (CVID-C (n = 24)) and (2) those with only infectious symptoms (CVID-OI (n = 9)). Flow cytometry of peripheral blood was performed and compared with systemic lupus erythematosus (SLE) patients (n = 17) and healthy controls (n = 20). We found that all lymphocytes were lower in CVID-C and SLE. NK cells were lowest in CVID-C. Th17 cells were significantly reduced in CVID-C and SLE. Tregs were significantly lower in CVID-C and SLE. Bregs did not significantly differ between any groups. Class-switched memory B cells were significantly lower in CVID-C and CVID-OI. Lastly, plasmablasts were significantly higher in SLE. Among the T cell subsets, CVID-C patients had lower naive and recent thymic emigrant CD4+ T cells. In conclusion, reduced Treg, Th17, and NK cells are features of CVID with autoimmune complications, and class-switched memory B cells can help distinguish patients with different causes of autoimmunity. Future studies are needed to confirm whether reductions of Treg, Th17, and NK cells might be a biomarker of more complicated CVID cases.

scholarly journals High levels of gut-homing immunoglobulin A–positive+B lymphocytes support the pathogenic role of intestinal mucosal hyperresponsiveness in immunoglobulin A nephropathy patients

2020 ◽  
Author(s):  
Fabio Sallustio ◽  
Claudia Curci ◽  
Nada Chaoul ◽  
Giulia Fontò ◽  
Gabriella Lauriero ◽  
...  
Keyword(s):  
B Cells ◽  
B Lymphocytes ◽  
Immunoglobulin A ◽  
Serum Levels ◽  
Memory B Cells ◽  
Immunoglobulin A Nephropathy ◽  
Pathogenic Role ◽  
Significant Difference ◽  
Activated B Cells

Abstract Background Immunoglobulin A nephropathy (IgAN) is the most frequent primary glomerulonephritis. The role of the microbiota and mucosal immunity in the pathogenesis of IgAN remains a key element. To date, the hypothetical relationship between commensal bacteria, elevated tumour necrosis factor (TNF) superfamily member 13 [also known as B-cell activating factor (BAFF)] levels, perturbed homoeostasis of intestinal-activated B cells and intestinal IgA class switch has not been clearly shown in IgAN patients. Methods We studied the intestinal–renal axis connections, analysing levels of BAFF, TNF ligand superfamily member 13 (APRIL) and intestinal-activated B cells in IgAN patients, healthy subjects (HSs) and patients with non-IgA glomerulonephritides. Results IgAN patients had increased serum levels of BAFF cytokine, correlating with higher amounts of five specific microbiota metabolites, and high APRIL cytokine serum levels. We also found that subjects with IgAN have a higher level of circulating gut-homing (CCR9+ β7 integrin+) regultory B cells, memory B cells and IgA+ memory B cells compared with HSs. Finally, we found that IgAN patients had high levels of both total plasmablasts (PBs) and intestinal-homing PBs. Interestingly, PBs significantly increased in IgAN but not in patients with other glomerulonephritides. Conclusions Our results demonstrate a significant difference in the amount of intestinal-activated B lymphocytes between IgAN patients and HSs, confirming the hypothesis of the pathogenic role of intestinal mucosal hyperresponsiveness in IgAN. The intestinal–renal axis plays a crucial role in IgAN and several factors may contribute to its complex pathogenesis and provide an important area of research for novel targeted therapies to modulate progression of the disease.

CD21low B cells in common variable immunodeficiency do not show defects in receptor editing, but resemble tissue-like memory B cells

Blood ◽  
2010 ◽  
Vol 116 (18) ◽  
pp. 3682-3683 ◽  
Author(s):  
Mirzokhid Rakhmanov ◽  
Sylvia Gutenberger ◽  
Baerbel Keller ◽  
Michael Schlesier ◽  
Hans-Hartmut Peter ◽  
...  
Keyword(s):  
B Cells ◽  
Common Variable Immunodeficiency ◽  
Memory B Cells ◽  
Receptor Editing ◽  
Common Variable

Absence of Memory B Cells in Patients with Common Variable Immunodeficiency

2002 ◽  
Vol 103 (1) ◽  
pp. 34-42 ◽  
Author(s):  
Kazunaga Agematsu ◽  
Takeshi Futatani ◽  
Sho Hokibara ◽  
Norimoto Kobayashi ◽  
Masaya Takamoto ◽  
...  
Keyword(s):  
B Cells ◽  
Common Variable Immunodeficiency ◽  
Memory B Cells ◽  
Common Variable

G1/260 – Correlation between switched memory B cells and development of bronchietasis in patients with Common variable immunodeficiency

2006 ◽  
Vol 7 ◽  
pp. S306-S307
Author(s):  
Asghhar Aghamohammadi ◽  
Mostafa Moin ◽  
Mohammad Vodjgani ◽  
Morteza Samadi ◽  
Abdolfattah Sarraf Nejad ◽  
...  
Keyword(s):  
B Cells ◽  
Common Variable Immunodeficiency ◽  
Memory B Cells ◽  
Common Variable

The loss of IgM memory B cells correlates with clinical disease in common variable immunodeficiency

2005 ◽  
Vol 115 (2) ◽  
pp. 412-417 ◽  
Author(s):  
Rita Carsetti ◽  
Maria Manuela Rosado ◽  
Simona Donnanno ◽  
Vanessa Guazzi ◽  
Annarosa Soresina ◽  
...  
Keyword(s):  
B Cells ◽  
Common Variable Immunodeficiency ◽  
Memory B Cells ◽  
Clinical Disease ◽  
Common Variable
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