Evans syndrome

When autoantibodies attack healthy red blood cells, platelets, and neutrophils (white blood cells), depleting the levels of blood cells necessary for normal functioning. Specifically, this targeting results in the development of autoimmune hemolytic anemia (AIHA), idiopathic thrombocytopenic purpura (ITRP), and (less commonly) neutropenia.

Anemia, fatigue, pallor, lightheadedness, shortness of breath, darker urine, rapid heartbeat, jaundice, petechiae (pinpoint, round spots that appear on the skin as a result of bleeding), larger areas of bruising, rashes with purple spotting (purpura), increased susceptibility to recurrent infections, fevers, malaise, ulcers on the mouth’s mucous membranes, and enlargement of the lymph nodes, spleen, and liver. If untreated, sepsis, hemorrhaging episodes, and significant cardiovascular problems can also arise.

Autoimmune hemolytic anemia, idiopathic thrombocytopenic purpura, and neutropenia, ecchymosis, autoimmune lymphoproliferative syndrome (ALPS), lupus, antiphospholipid syndrome, Sjogren’s syndrome, common variable immunodeficiency, IgA deficiency, certain lymphomas, and chronic lymphocytic leukemia.

Some studies suggest that Evans syndrome is more frequently seen in children, though it can arise at any age. Infections, cancerous growths, autoimmune conditions, certain vaccinations, drugs, or, rarely, a sibling with Evans syndrome when both have congenital heart defects and other inherited disorders.

• COVID-19-associated Evans syndrome: A case report and review of the literature (2022)
• Pediatric Evans Syndrome: A 20-year experience from a tertiary center in Brazil (2023)
• T-follicular helper cell expansion and chronic T-cell activation are characteristic immune anomalies in Evans syndrome (2022)
• Underlying Inborn Errors of Immunity in Patients With Evans Syndrome and Multilineage Cytopenias: A Single-Centre Analysis (2022)