Farzaneh Tofighi Zavareh1,2,3, Abbas Mirshafiey1,2, Reza Yazdani1, Abbas Ali Keshtkar4, Hassan Abolhassani1,5, Yasser Bagheri6, Arezou Rezaei1, Samaneh Delavari1, Nima Rezaei1,3,7, Asghar Aghamohammadi1. 1. Research Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran. 2. Department of Immunology, School of Public Health, Tehran University of Medical Sciences, Tehran, Iran. 3. Primary Immunodeficiency Diseases Network (Pidnet), Universal Scientific Education and Research Network (USERN), Tehran, Iran. 4. Department of Health Sciences Education Development, School of Public Health, Tehran University of Medical Sciences, Tehran, Iran. 5. Division of Clinical Immunology, Department of Laboratory Medicine, Karolinska Institute at Karolinska University Hospital Huddinge, Stockholm, Sweden. 6. Clinical Research Development Unit (CRDU), 5 Azar Hospital, Golestan University of Medical Sciences, Gorgan, Iran. 7. Department of Immunology, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran.
Abstract
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 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.
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 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.
Entities:
Keywords:
Primary immunodeficiency; clinical profile; common variable immunodeficiency (CVID); inborn errors of immunity; lymphocytes subsets; proliferation response
Authors: Aleksandra Szczawińska-Popłonyk; Katarzyna Ta Polska-Jóźwiak; Eyal Schwartzmann; Natalia Popłonyk Journal: Front Pediatr Date: 2022-03-23 Impact factor: 3.418