Evan’s syndrome, primary or secondary? Navigating the diagnostic puzzle

Abstract

Evans syndrome (ES) is a rare autoimmune disorder characterized by the simultaneous or sequential occurrence of autoimmune hemolytic anemia (AIHA) and immune thrombocytopenic purpura (ITP). Distinguishing between primary and secondary forms is crucial for management. This report presents a 69-year-old male with bleeding gums, anemia, and thrombocytopenia, who was diagnosed with ES following an extensive workup. Multiple potential triggers, including recent vaccinations, antibiotic use, past tuberculosis, and skin malignancies, were identified, highlighting the multifactorial nature of ES. The patient was treated with glucocorticoids, intravenous immunoglobulin (IVIG), Rituximab, and Romiplostim resulting in improved hematologic parameters. This case underscores the diagnostic challenges and importance of individualized therapeutic approaches in managing ES. Understanding the potential triggers and underlying immune dysregulation is pivotal for accurate diagnosis and effective treatment.