Lymphocytes Subsets In Correlation With Clinical Profile In Cvid Patients Without Monogenic Defects

Objectives: 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. Methods: 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. 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 CD21(low) B and terminally differentiated effector memory (T-EMRA) 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 CD21(low) B and T-EMRA 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.