Prevalence of Anti-lymphocyte Antibodies in Patients with Good Syndrome and Partial RAG Deficiency

Abstract

Good syndrome (GS) is a rare adult-onset progressive combined immunodeficiency typically characterized by thymoma, hypogammaglobulinemia and variable CD4+ lymphocytopenia. Of note, opportunistic infections and autoimmune complications have been increasingly recognized in these patients, implying underlying immune dysregulation. Autoantibody profiling, including the prevalence of antibodies targeting cytokines (associated with thymoma) and lymphocytes (associated with pathogenic lymphocytopenia) remains unclear.