An Overview of Evans Syndrome–A Rare Disease

Evans syndrome is characterized by the simultaneous or consecutive occurrence of immune thrombocytopenia, warm autoimmune hemolytic anemia, and, less frequently, autoimmune neutropenia. It is linked to non-cross-reacting autoantibodies that target antigens unique to neutrophils, platelets, or red blood cells.Corticosteroids or immunoglobulins are the first-line treatment for Evans syndrome. For patients who are not responsive to steroids, rituximab or splenectomy are the second-line treatments. Deficits of CTLA-4, LRBA, TPP2, and a reduced CD4/CD8 ratio are among the recent molecular ideas that explain the physiopathology of ES .Rituximab, mofetil mycophenolate, cyclosporine, vincristine, azathioprine, sirolimus, and thrombopoietin receptor agonists are among the second-line treatments for refractory ES. Hematopoietic stem cell transplantation has been effective in situations where immunosuppressive medications have failed to work. Since ES is chronic and has a high recurrence rate despite improvements, timely diagnosis, cautious treatment, and close patient monitoring are necessary to improve quality of life and achieve the best results. Prospective clinical trials are required for possible targeted therapy in order to improve ES.