Dysregulated B cell function in Ataxia-telangiectasia patients with IgA deficiency

Background Patients with Ataxia-telangiectasia (A-T) suffer from progressive cerebellar ataxia, immunodeficiency, respiratory failure, and cancer susceptibility. Deficiencies in both, cellular and humoral immunity, including IgA deficiency caused by a disturbed B- and T cell homeostasis have been described. Overall survival of patients with IgA deficiency is significantly diminished. The aim of this study was to elucidate the epigenetic dysregulation in B cell function in A-T patients with IgA deficiency and the background of abnormal B-cell function in patients with IgA deficiency by mimicking the germline center environment.