Evans Syndrome

Evans syndrome is a rare autoimmune disease of the blood where the body’s own immune system attacks its red blood cells and platelets. It is usually associated with autoimmune haemolytic anaemia (AIHA) and immune thrombocytopenia (ITP), and in certain cases autoimmune neutropenia may also be present. The syndrome can occur in children and adults but is considered rare.
Symptoms depend on which blood cells are impacted. The destruction of red blood cells can lead to weariness, weakness, shortness of breath, pale skin, and jaundice. When platelet counts are low, easy bruising, nosebleeds, bleeding from the gums, and persistent bleeding from minor injuries can occur. The symptoms tend to come back.

The aetiology of Evans Syndrome is typically unknown, but it can be related to other autoimmune diseases, immunological deficiency, or lymphoproliferative disorders. Diagnosis is by blood tests such as complete blood count, reticulocyte count, direct antiglobulin (Coombs) test, and evaluation of underlying diseases.
Treatment is aimed at regulating the immunological response and the death of blood cells. First-line therapy commonly includes corticosteroids and intravenous immunoglobulin (IVIG). Immunosuppressive drugs, biologic treatments, or splenectomy may be required in patients with an inadequate response. With regular monitoring and tailored treatment, many patients experience symptom relief and better quality of life.