Paucity of gastrointestinal plasma cells in common variable immunodeficiency

Area of Science:

• Gastroenterology
• Immunology
• Pathology

Background:

• Common variable immunodeficiency enteropathy (CVID-E) is a noninfectious GI complication of CVID.
• A paucity of plasma cells is a frequent feature of CVID and CVID-E, but lacks standardized diagnostic criteria.
• Histopathological analysis of plasma cells in GI biopsies for CVID-E is inconsistent.

Purpose of the Study:

• To systematically review methods for reproducible plasma cell quantification in CVID biopsies.
• To describe plasma cell counts and classes reported in the literature for CVID and CVID-E.
• To propose standardized methodologies for plasma cell quantification in GI biopsies.

Main Methods:

• Systematic literature review of studies reporting plasma cell quantification in CVID and CVID-E.
• Analysis of reported plasma cell counts and classes in gastrointestinal biopsies.
• Evaluation of methodologies for plasma cell quantification.

Main Results:

• Reduced plasma cell counts are prevalent throughout the GI tract, excluding the esophagus.
• Immunoglobulin A+ (IgA+) plasma cells are the most commonly reduced type in CVID.
• Limited literature exists on the predictive value of low IgA+ plasma cell counts in CVID-E.

Conclusions:

• Standardized quantification of plasma cells is crucial for CVID-E diagnosis and research.
• Proposed methodologies include <10 plasma cells/HPF or ≥1-5% of mononuclear cells.
• These definitions require analysis over ≥3 sections and ≥2 biopsies for diagnostic consistency.