What condition is characterized by early onset hypogammaglobulinemia, autoimmunity, and immune dysregulation?

The condition characterized by early onset hypogammaglobulinemia, autoimmunity, and immune dysregulation is LRBA deficiency. This is a primary immunodeficiency disorder that results from mutations in the LRBA gene, which plays a critical role in the regulation of immune responses.

LRBA deficiency typically manifests in childhood with symptoms that include recurrent infections due to low levels of immunoglobulins (hypogammaglobulinemia). In addition to recurrent infections, individuals with LRBA deficiency often experience autoimmune manifestations. The dysregulation of the immune system in this condition can lead to various autoimmune diseases, where the immune system mistakenly attacks healthy tissues.

While other options like lupus, common variable immune deficiency, and severe combined immunodeficiency are associated with immune issues, they do not specifically present the triad of early onset hypogammaglobulinemia, autoimmunity, and immune dysregulation in the same way as LRBA deficiency. Lupus is primarily an autoimmune disease with a complex etiology, common variable immune deficiency is characterized by recurrent infections and low immunoglobulins but does not typically present with significant autoimmune components early on, and severe combined immunodeficiency affects both T and B cell function but usually