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

Common variable immunodeficiency (CVID) patients experience clinical manifestations rather than recurrent respiratory infections including autoimmunity, enteropathy, and lymphoproliferation. We evaluated the correlation of lymphocyte subpopulations with such manifestations. Twenty-six genetically unsolved CVID patients were subdivided into four phenotypes: infection only (IO), autoimmunity (AI), chronic enteropathy (CE), and lymphoproliferative disorders (LP) and examined for lymphocyte subsets by flow cytometry and TCD4 proliferation by Carboxyfluorescein succinimidyl ester (CFSE) test. 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 CD21 B and terminally differentiated effector memory (T) 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. In genetically unsolved CVID patients, increased Th2 and Th17 and reduced Treg is associated with IO, increased CD21 B and T 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.