scholarly journals Association of B-cell activating factor receptor deficiency with the P21R polymorphism and common variable immunodeficiency

2015 ◽  
Vol 115 (1) ◽  
pp. 82-83 ◽  
Author(s):  
Matthew Germinaro ◽  
Paul Reynolds ◽  
Vijaya Knight ◽  
Rafeul Alam
Keyword(s):  
B Cell ◽  
Common Variable Immunodeficiency ◽  
B Cell Activating Factor ◽  
Common Variable

scholarly journals Serum B cell activating factor and interleukin 10 levels in common variable immunodeficiency: Relationship with clinical findings

2019 ◽  
Vol 76 (1) ◽  
pp. 42-49
Author(s):  
Radovan Mijanovic ◽  
Sladjana Andrejevic ◽  
Vladimir Jurisic ◽  
Branka Bonaci-Nikolic
Keyword(s):  
Autoimmune Diseases ◽  
B Cell ◽  
Common Variable Immunodeficiency ◽  
Healthy Subjects ◽  
Serum Levels ◽  
Pulmonary Diseases ◽  
Recurrent Infections ◽  
B Cell Activating Factor ◽  
Chronic Pulmonary Diseases ◽  
Common Variable

Background/Aim. Common variable immunodeficiency (CVID) is an immunologically and clinically heterogeneous disorder. Disturbed cytokine production is implicated in dysfunctional immune response. The aim of this study was to investigated B-cell activating factor (BAFF) and interleukin (IL)- 10 levels in CVID patients. Methods. The study included 28 CVID patients diagnosed and followed during a 20-year period (mean follow-up 14.5 years). Control groups consisted of 4 patients with X-linked agammaglobulinemia (XLA) and 21 healthy subjects. According to clinical characteristics, the CVID patients were divided into four groups which partly overlap: chronic pulmonary diseases (n = 21), splenomegaly (n = 13), autoimmune diseases (n = 9) and patients with recurrent infections despite regular intravenous immunoglobulin (IVIg) substitution (n = 4). The serum levels of BAFF and IL-10 were measured by commercial ELISA. Results. The BAFF levels were found to be higher in all CVID patients compared to the healthy controls (p < 0.01). The most significant differences were observed in the patients with pulmonary diseases and splenomegaly (p < 0.0001). Also, concentrations of IL-10 were higher in all CVID patients in comparison with the XLA patients (p < 0.05) and healthy subjects (p < 0.01). A statistically significant positive correlation (r = 0.86; p < 0.01) was found between the levels of BAFF and IL-10 in the CVID patients with autoimmune diseases. We demonstrated that the CVID patients with chronic pulmonary diseases had higher levels of IL-10, while the CVID patients with recurrent infections had higher BAFF concentrations in comparison to the patients without these features (p < 0.05). Conclusion. In spite of the limited number of patients, this is the first report from Serbia, examining the serum levels of BAFF and IL-10 in the CVID patients. Our study showed significantly increased concentrations of serum BAFF and IL-10 in the patients with CVID compared to the healthy subjects. Further studies are needed to confirm our findings that the BAFF levels are more pronounced in patients with recurrent infections while IL-10 levels are higher in patients with chronic pulmonary diseases.

scholarly journals Changes in B cell immunophenotype in common variable immunodeficiency: cause or effect - is bronchiectasis indicative of undiagnosed immunodeficiency?

2013 ◽  
Vol 171 (2) ◽  
pp. 195-200 ◽  
Author(s):  
P. Bright ◽  
S. Grigoriadou ◽  
P. Kamperidis ◽  
M. Buckland ◽  
A. Hickey ◽  
...  
Keyword(s):  
B Cell ◽  
Common Variable Immunodeficiency ◽  
Common Variable

T and B lymphocyte abnormalities in bone marrow biopsies of common variable immunodeficiency

Blood ◽  
2011 ◽  
Vol 118 (2) ◽  
pp. 309-318 ◽  
Author(s):  
Manuella L. Gomes Ochtrop ◽  
Sigune Goldacker ◽  
Annette M. May ◽  
Marta Rizzi ◽  
Ruth Draeger ◽  
...  
Keyword(s):  
Bone Marrow ◽  
T Cell ◽  
B Cell ◽  
Common Variable Immunodeficiency ◽  
Plasma Cells ◽  
Cell Development ◽  
B Cell Development ◽  
Bone Marrow Biopsies ◽  
Cell Counts ◽  
Common Variable

Abstract In common variable immunodeficiency (CVID) defects in early stages of B-cell development, bone marrow (BM) plasma cells and T lymphocytes have not been studied systematically. Here we report the first morphologic and flow cytometric study of B- and T-cell populations in CVID BM biopsies and aspirates. Whereas the hematopoietic compartment showed no major lineage abnormalities, analysis of the lymphoid compartment exhibited major pathologic alterations. In 94% of the patients, BM plasma cells were either absent or significantly reduced and correlated with serum immunoglobulin G levels. Biopsies from CVID patients had significantly more diffuse and nodular CD3+ T lymphocyte infiltrates than biopsies from controls. These infiltrates correlated with autoimmune cytopenia but not with other clinical symptoms or with disease duration and peripheral B-cell counts. Nodular T-cell infiltrates correlated significantly with circulating CD4+CD45R0+ memory T cells, elevated soluble IL2-receptor and neopterin serum levels indicating an activated T-cell compartment in most patients. Nine of 25 patients had a partial block in B-cell development at the pre-B-I to pre-B-II stage. Because the developmental block correlates with lower transitional and mature B-cell counts in the periphery, we propose that these patients might form a new subgroup of CVID patients.

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