Efgartigimod as a Steroid-sparing Therapy for Evans Syndrome: A Case Report

Evans syndrome is characterized by the coexistence of immune thrombocytopenia (ITP) and autoimmune hemolytic anemia (AIHA). Corticosteroids are the first-line therapy. However, many patients become steroid-dependent or experience significant adverse effects. An 80-year-old man with a 33-year history of ITP was admitted with laboratory findings suggestive of hemolytic anemia and he was diagnosed with Evans syndrome. High-dose corticosteroids achieved hematologic control, but they also caused delirium, necessitating dose reduction. Rituximab produced a limited response. Efgartigimod was initiated for chronic ITP with the expectation of a concurrent benefit for AIHA. Following treatment, the platelet counts improved, hemolysis stabilized, and corticosteroids were successfully tapered without relapse. This case suggests that efgartigimod may be effective in IgG-mediated autoimmune cytopenias, such as Evans syndrome, and it may serve as a steroid-sparing therapeutic option.