Evans Syndrome

What is Evans syndrome?

Evans syndrome is an autoimmune disorder in which antibodies attack red blood cells (RBC), which deliver oxygen to body tissues, platelets (which help blood clot), and/or neutrophils (a type of white blood cell). The disorder resembles a combination of autoimmune hemolytic anemia (the destruction of young red blood cells), thrombocytopenia (too few platelets), and neutropenia (too few neutrophils). These may occur simultaneously or one may follow the other.

There is no known genetic cause of Evans syndrome, and occurrences in families are rare. It is considered a chronic disease, with periods of remission and exacerbation. Thrombocytopenia recurs in about 60 percent of patients, while autoimmune hemolytic anemia (AIHA) recurs in about 30 percent.

What are the symptoms of Evans syndrome?

The symptoms of Evans syndrome may be similar in nature to leukemia and lymphoma; these illnesses must be ruled out before a diagnosis is made.

If a child has a low red blood cell (RBC) count, symptoms may include:

If a child has a low platelet count, symptoms may include:

  • increased bruising
  • petechia: tiny red dots under the skin that are a result of very small bleeds into the skin
  • increased bleeding symptoms, such as a bloody nose or heavy menses

If a child has a low neutrophil count, symptoms may include:

  • fevers
  • mouth sores
  • increased bacterial infections

What causes Evans syndrome?

The current cause for Evans syndrome is unknown.

How we care for Evans syndrome

Children with Evans syndrome are treated at Dana-Farber/Boston Children’s Cancer and Blood Disorders Center. Our Blood Disorders Center provides comprehensive diagnostic, treatment, and preventive services for a wide range of non-malignant blood disorders, including Evans syndrome. Your child will receive care from some of the world’s most experienced hematologists.