Evans syndrome

 
 

Overview

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.

Common Symptoms

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.

Coexisting Diseases and Conditions

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.

Risk Factors and Prevalence

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.

Sources

  1. Article Sources and Footnotes

    1. Autoimmune hemolytic anemia | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program. (n.d.). Retrieved July 12, 2021, from https://rarediseases.info.nih.gov/diseases/5870/autoimmune-hemolytic-anemia

    2. Evans Syndrome. (n.d.). NORD (National Organization for Rare Disorders). Retrieved July 12, 2021, from https://rarediseases.org/rare-diseases/evans-syndrome/

    3. Immune thrombocytopenia (ITP)—Symptoms and causes. (n.d.). Mayo Clinic. Retrieved July 12, 2021, from https://www.mayoclinic.org/diseases-conditions/idiopathic-thrombocytopenic-purpura/symptoms-causes/syc-20352325

    4. Jaime-Pérez, J. C., Aguilar-Calderón, P. E., Salazar-Cavazos, L., & Gómez-Almaguer, D. (2018). Evans syndrome: Clinical perspectives, biological insights and treatment modalities. Journal of Blood Medicine, 9, 171–184. https://doi.org/10.2147/JBM.S176144