Paucity of gastrointestinal plasma cells in common variable immunodeficiency

Common variable immunodeficiency enteropathy (CVID-E) is a noninfectious complication of CVID caused by chronic inflammation of the gastrointestinal (GI) tract. Based on literature, a paucity or lack of plasma cells, although not obligatory for diagnosis, is a pathognomonic feature of CVID and more frequent in CVID-E. However, there is no consensus on standardized histopathological analysis of this feature in biopsies. In this systematic review, we highlight methods of reproducible plasma cell quantification of biopsies in CVID and describe the plasma cell counts and classes as presented in the literature.

Reduced plasma cell counts are commonly found over the entire GI tract, except for in the oesophagus. Immunoglobulin A+ (IgA+) plasma cells appear to be the most commonly reduced plasma cell class in CVID, yet there is scarce literature on the predictive value of low IgA+ plasma cell counts in CVID-E.

We propose two optimized methodologies of quantification using a cut-of value of <10 plasma cells per HPF at 40× magnification, or a proportion of ≥1-5% of total mononuclear cells, recorded over ≥3 sections, and in ≥2 biopsies, as the most conservative agreeable definitions for a paucity of plasma cells to be used in diagnostics and further research.