CD21-/low B Cells Are Increased in Both Common Variable Immune Deficiency (CVID) and Specific Antibody Deficiency(SAD)

2011 ◽  
Vol 127 (2) ◽  
pp. AB135-AB135 ◽  
Author(s):  
A. Elizalde ◽  
S. Hudey ◽  
M. Dorsey ◽  
E. Perez ◽  
J. Sleasman ◽  
...  
Keyword(s):  
Immune Deficiency ◽  
B Cells ◽  
Specific Antibody ◽  
Antibody Deficiency ◽  
Common Variable Immune Deficiency ◽  
Specific Antibody Deficiency ◽  
Common Variable

Bronchiectasis severity correlates with outcome in patients with primary antibody deficiency

Thorax ◽  
2021 ◽  
pp. thoraxjnl-2020-215585
Author(s):  
Jimstan Periselneris ◽  
Silke Schelenz ◽  
Michael Loebinger ◽  
Patricia Macedo ◽  
Zoe Adhya ◽  
...  
Keyword(s):  
Immune Deficiency ◽  
Pseudomonas Aeruginosa ◽  
Lung Disease ◽  
Specific Antibody ◽  
Antibody Deficiency ◽  
Primary Antibody ◽  
Common Variable Immune Deficiency ◽  
Primary Antibody Deficiency ◽  
Tertiary Referral ◽  
Common Variable

Bronchiectasis is a well-recognised complication of primary antibody deficiency (PAD) syndromes. Previous data suggest that mortality in common variable immune deficiency (CVID) is not associated with isolated bronchiectasis. A retrospective analysis of patients with CVID and specific antibody deficiency in two tertiary referral centres with lung disease was conducted. Severity of bronchiectasis at presentation was associated with mortality. Lower FEV1, colonisation with Pseudomonas aeruginosa and a diagnosis of COPD were also associated with mortality. Bronchiectasis is an important driver of mortality in patients with PAD syndromes.

scholarly journals Role of B cells in common variable immune deficiency

2009 ◽  
Vol 5 (5) ◽  
pp. 557-564 ◽  
Author(s):  
Sam Ahn ◽  
Charlotte Cunningham-Rundles
Keyword(s):  
Immune Deficiency ◽  
B Cells ◽  
Common Variable Immune Deficiency ◽  
Common Variable

A case of Common Variable Immune Deficiency with Lung disease- Not Just Bronchiectasis

2021 ◽  
Author(s):  
sharon polakow farkash
Keyword(s):  
Immune Deficiency ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Pediatric Population ◽  
Genetic Diagnosis ◽  
Immune Dysregulation ◽  
Antibody Deficiency ◽  
Response To Treatment ◽  
Common Variable Immune Deficiency ◽  
Common Variable

Introduction: Common Variable Immune Deficiency (CVID) is the most prevalent form of severe antibody deficiency in children and in adults. Most patients have recurrent infections, mainly sinopulmonary infections. Despite adequate IVIG replacement therapy chronic lung disease continues to be a main cause of morbidity and mortality. The term granulomatous-lymphocytic interstitial lung disease (GLILD) is frequently used to described the Interstitial lung disease associated with immune dysregulation in primary antibody deficiency such as CVID. Aim: To describe a 10-year-old boy with CVID who developed GLILD and his response to treatment with Rituximab. Discussion: Although GLILD is a well described condition that accompanies CVID as a manifestation of immune dysregulation, it is still under recognized, especially in the pediatric population. Among experts, there is little uniformity when it comes to diagnostic and treatment approaches. Recent studies showed improved outcomes when using combination therapy with Rituximab, such as in our case presentation. Statement of Novelty: This report discusses a case of CVID in a 10-year-old boy, with no genetic diagnosis, whose lung functions and general condition continued to deteriorate despite adequate IGRT and MMF treatment. After the diagnosis of GLILD we initiated treatment with a 4-dose weekly course of Rituximab with prompt resolution of his interstitial disease. In our case we shed light on GLILD, an important condition that accompanies CVID and demonstrate an excellent response to Rituximab-a steroid sparing agent, which is a crucial aspect when considering therapeutic choices for the pediatric population.

scholarly journals TACI deficiency impairs sustained Blimp-1 expression in B cells decreasing long-lived plasma cells in the bone marrow

Blood ◽  
2011 ◽  
Vol 118 (22) ◽  
pp. 5832-5839 ◽  
Author(s):  
Shoichiro Tsuji ◽  
Catarina Cortesão ◽  
Richard J. Bram ◽  
Jeffrey L. Platt ◽  
Marilia Cascalho
Keyword(s):  
Immune Deficiency ◽  
B Cells ◽  
Plasma Cells ◽  
Common Variable Immune Deficiency ◽  
Dna Double Strand Breaks ◽  
Protein Antigens ◽  
Strand Breaks ◽  
First Time ◽  
Common Variable ◽  
Antibody Secreting Cells

Abstract Deficiencies in transmembrane activator and CAML interactor (TACI) result in common variable immune deficiency, a syndrome marked by recurrent infections with encapsulated microorganisms, impaired production of antibodies, and lymphoproliferation. How TACI promotes antibody production and inhibits lymphoproliferation is not understood. To answer this question, we studied the generation of immunity to protein antigens in both TACI-deficient and TACI-proficient mice. We show that TACI promotes sustained Blimp-1 expression by B cells responding to antigen, which in turn limits B-cell clonal expansion and facilitates differentiation of long-lived antibody-secreting cells. Short-term IgG secretion occurs independently of TACI as DNA double-strand breaks associated with isotype class switching induce Blimp-1 transiently, independently of TACI. Our results showing that TACI induces and maintains Blimp-1 provide, for the first time, a unified molecular and cellular mechanism explaining the primary features of common variable immune deficiency, exquisite vulnerability to infection with encapsulated organisms, lymphoproliferation, and hypogammaglobulinemia.

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