Role of rare immune cells in common variable immunodeficiency

Abstract

Common variable immunodeficiency disorder (CVID) is the most frequent symptomatic inborn errors of immunity (IEIs) 1 with an estimated prevalence of 1:50,000-1:25,000. 2 CVID is characterized by hypogammaglobulinemia, impaired production of specific immunoglobulin (Ig), and increased susceptibility to recurrent infections. Clinical manifestations in CVID patients demonstrate a wide spectrum of complications including recurrent infections, pulmonary complications, granulomatous or polyclonal lymphocytic infiltrations, autoimmunity, gastrointestinal diseases, and neoplasia, which can be established in different periods of life, from childhood to late adulthood. 3,4 Immunoglobulin replacement therapy is the most important therapeutic intervention for decreasing chronic infections and infectious sequels of these patients. 5