scholarly journals 1316. Uncommon Presentations of Common Variable Immunodeficiency

2021 ◽  
Vol 8 (Supplement_1) ◽  
pp. S746-S747
Author(s):  
Akankcha Alok ◽  
John Greene ◽  
Sadaf Aslam

Abstract Background Common Variable Immunodeficiency (CVID) is a primary immunodeficiency disorder which affects B lymphocyte function and differentiation causing decreased levels of Immunoglobulin G (IgG), Immunoglobulin A (IgA) and Immunoglobulin M (IgM).1 The objective of this study is to highlight how hypogammaglobulinemia can lead to respiratory infections with microbes that are lesser known in the background of CVID with the help of a two-case series. Methods Medical records of two patients with CVID were reviewed who were found to have mycobacterium avium-complex intracellulare and streptococcus agalactiae lung infections respectively. Results Decreased IgG in CVID means reduced antibody production, low IgA leads to mucosal inflammation and increased susceptibility to respiratory infections2 and lower IgM memory B-cells causes infections with encapsulated microorganisms.3 Table 1 highlights the various respiratory infections and their etiologies that have been reported with CVID, the most common being encapsulated organisms like Haemophilus influenza, Streptococcus pneumonia, Neisseria meningitidis along with enterovirus. Table 2 demonstrates our findings. In the first case we have reported a patient with mycobacterium avium-complex intracellulare (MAC-I). This could be because of hypogammaglobulinemia, decreased B and T-cell interaction and reduced T-cell signaling caused by CVID.4 Although, mycobacterium tuberculosis, simiae and hominis lung infections and mycobacterium bovis systemic infections have been reported before, MAC-I is relatively rare in CVID.5 In our second case, the patient developed streptococcus agalactiae or Group-B streptococcus (GBS) empyema. Most cases of GBS have been reported in pregnant women and infants. Infections with other encapsulated organisms have been reported in CVID but GBS empyema is less frequent and can happen due to decreased bacteria-specific CD4 cells, microbial translocation and hypogammaglobulinemia.6 . Table 1. Respiratory Infections reported in CVID along with their etiologies. Figure 1. CT image of MAC-I infection. Conclusion We encountered two unique cases of CVID with rare infectious etiologies. The cases are intended to create an awareness and vigilance regarding CVID induced hypogammaglobulinemia which can cause respiratory infections with lesser known pathogens where antibodies may be important. Disclosures All Authors: No reported disclosures

2018 ◽  
Vol 6 (1) ◽  
pp. 159-168.e3 ◽  
Author(s):  
Johannes M. Sperlich ◽  
Bodo Grimbacher ◽  
Sarita Workman ◽  
Tanzina Haque ◽  
Suranjith L. Seneviratne ◽  
...  

2010 ◽  
Vol 126 (3) ◽  
pp. 671-675 ◽  
Author(s):  
Nagaja Capitani ◽  
Amedeo Amedei ◽  
Silvia Rossi Paccani ◽  
Andrea Matucci ◽  
Alessandra Vultaggio ◽  
...  

2000 ◽  
Vol 30 (9) ◽  
pp. 2632-2638 ◽  
Author(s):  
Marianna Boncristiano ◽  
M. Bernardetta Majolini ◽  
Mario M. D'Elios ◽  
Sonia Pacini ◽  
Silvia Valensin ◽  
...  

1993 ◽  
Vol 33 ◽  
pp. S24-S28 ◽  
Author(s):  
Jon S Jaffe ◽  
Eli Eisenstein ◽  
Michael C Sneller ◽  
Warren Strober

Blood ◽  
2011 ◽  
Vol 118 (2) ◽  
pp. 309-318 ◽  
Author(s):  
Manuella L. Gomes Ochtrop ◽  
Sigune Goldacker ◽  
Annette M. May ◽  
Marta Rizzi ◽  
Ruth Draeger ◽  
...  

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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