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What is Evans syndrome

Evans syndrome is a very rare autoimmune disorder in which the immune system destroys the body’s red blood cells, white blood cells and/or platelets. Affected people often experience thrombocytopenia (too few platelets) and Coombs’ positive hemolytic anemia (premature destruction of red blood cells). Signs and symptoms may include purpura, paleness, fatigue, and light-headedness. The exact cause of this condition is unknown. The best treatment options for Evans syndrome depend on many factors, including the severity of the condition, the signs and symptoms present, and each person’s response to certain therapies.

Evans syndrome is a very rare autoimmune disorder in which the immune system destroys the body’s red blood cells, white blood cells and/or platelets. Affected people often experience thrombocytopenia (too few platelets) and Coombs’ positive hemolytic anemia (premature destruction of red blood cells). Signs and symptoms may include purpura, paleness, fatigue, and light-headedness. The exact cause of this condition is unknown. The best treatment options for Evans syndrome depend on many factors, including the severity of the condition; the signs and symptoms present; and each person’s response to certain therapies.[1][2]

The signs and symptoms of Evans syndrome vary from person to person and largely depend on which type(s) of blood cells are affected (i.e. red blood cells, platelets, and/or white blood cells). If a person does not have enough healthy red blood cells (anemia), they may experience weakness, fatigue, paleness, light-headedness, shortness of breath, or rapid heartbeat. Low platelets (thrombocytopenia) can cause easy or unexplained bruising; red or purple spots on the skin (petechiae); prolonged bleeding from small cuts; and purpura. People with a low white blood cell count (neutropenia) may be more susceptible to infections, and may experience symptoms such as fever or sores inside the mouth.[2]

Many people with Evans syndrome go through periods of remission in which the signs and symptoms of the condition temporarily disappear or become less severe (usually induced temporarily by treatment).[2]

This information is provided by the National Institutes of Health (NIH) Genetic and Rare Diseases Information Center (GARD).
https://rarediseases.info.nih.gov/diseases/6389/evans-syndrome

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