Evans syndrome is an autoimmune disorder in which a person’s own 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 that helps fight infections). The disorder resembles a combination of autoimmune hemolytic anemia (AIHA; the premature destruction of RBC), thrombocytopenia (too few platelets), and/or 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.
Children with Evans syndrome are treated at Dana-Farber/Boston Children’s Cancer and Blood Disorders Center through our Blood Disorders Center. Continue reading to learn more about Evans syndrome or visit the Blood Disorders Center homepage to learn about our expertise.
The symptoms of Evans syndrome may be similar in nature to leukemia and lymphoma, so those
illnesses must be ruled out before a diagnosis is made. If a child has a low
RBC count, symptoms may include:
If a child has a low platelet count, symptoms may include:
If a child has a low neutrophil count, symptoms may include:
Doctors diagnose Evans syndrome with a variety of blood tests, including:
Other tests may include bone marrow aspiration
and biopsy and flow cytometry, a technique for identifying and sorting cells by
staining with a fluorescent dye. After all tests are completed, doctors will be
able to outline the best treatment options.
The first-line treatments for Evan’s syndrome are corticosteroid, such as prednisone, and intravenous immunoglobulin, which help to control acute episodes. Most children respond to this treatment, but relapses frequently occur. Other treatments include: