Lymphocytes subsets in correlation with clinical profile in CVID patients without monogenic defects

Abstract

Objectives Common variable immunodeficiency (CVID) patients experience clinical manifestations rather than recurrent respiratory infections including autoimmunity, enteropathy and lymphoproliferation. We evaluated correlation of lymphocyte subpopulations with such manifestations. Methods: Twenty-six genetically unsolved CVID patients subdivided into 4 phenotypes: infection only (IO), autoimmunity (AI), chronic enteropathy (CE) and lymphoproliferative disorders (LP) were examined for B and T lymphocyte by flow cytometry and TCD4+ proliferation by Carboxyfluorescein succinimidyl ester (CFSE) test. Results We detected reduced memory B and increased total, Effector Memory (EM), cytotoxic and activated TCD8+ in IO, AI and CE, decreased plasmablasts, total and naive TCD4+, Regulatory TCD4+ (Treg) and naive TCD8+ in IO and CE, elevated CD21low B and Terminally Differentiated Effector Memory (TEMRA) TCD8+ in IO and AI, increased helper T (Th2) and Th17 in IO, decreased Th1 in AI and defective total and naive B and Central Memory (CM) TCD4+ in CE. IO showed reduced TCD4+ proliferation response. Conclusions In genetically unsolved CVID patients, increased Th2 and Th17 and reduced Treg is associated with IO, increased CD21low B and TEMRA TCD8+ and reduced Th1 is contributed to AI and reduced total and naive B, CM TCD4+ and naive TCD8+ and expanded total TCD8+ is correlated with CE.