Evans Syndrome in the Adolescent Female

Evans syndrome (ES) describes an immune dysregulation syndrome manifesting as multilineage autoimmune cytopenias, most often autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP), but also autoimmune neutropenia (AIN) in some cases. Since Evans and colleagues first described this phenomenon in the 1940s–1950s, this syndrome of immune cytopenias is increasingly identified to be secondary to underlying systemic immune dysregulation rather than idiopathic pathology. Some of the most common pathologies now known to drive ES include autoimmune lymphoproliferative syndrome (ALPS), common variable immunodeficiency (CVID), and other systemic autoimmune diseases such as systemic lupus erythematosus (SLE), particularly in adolescent females. These pathologies share the commonality of a dysregulated immune system in which autoreactive lymphocytes are poorly controlled, ultimately predisposing to autoimmunity. Importantly, clinical outcomes for patients with ES can be optimized by targeting specific immune dysregulation pathologies, underscoring the need for thorough individualized diagnostic and treatment strategies.