• Autoimmune Hemolytic Anemia (AIHA) Overview

    Autoimmune hemolytic anemia (AIHA) is a rare red blood cell (RBC) disorder that occurs when antibodies directed against a person’s own RBC cause them to burst, leading to insufficient concentration in the blood. When this happens, the lifetime of RBC decreases from 100 to 120 days to just a few days in serious cases. Intracellular components of RBC are then released into the circulating blood and tissue, causing characteristic symptoms.

    The causes of autoimmune hemolytic anemia are poorly understood. It may be a primary disorder or secondary to an underlying illness, such as Epstein-Barr Virus, lymphoma, systemic lupus erythematosus, immunodeficiency disorders, rheumatoid arthritis, or ulcerative colitis. AIHA may also be a component of Evans Syndrome, an autoimmune disease that affects more than one blood cell line.

    Autoimmune Hemolytic Anemia Treatment at Dana-Farber/Boston Children’s

    Children with autoimmune hemolytic anemia are treated at Dana-Farber/Boston Children’s Cancer and Blood Disorders Center through our Rare Anemias and Iron Disorders Program. Continue reading to learn more about autoimmune hemolytic anemia or visit the Rare Anemias and Iron Disorders Program homepage to learn about our expertise and treatment options.

    Symptoms & Diagnosis

    The most common symptoms of autoimmune hemolytic anemia are:

    • Dark brown urine
    • Jaundice
    • Pale skin
    • Muscle pain
    • Headache
    • Nausea and vomiting
    • Weakness
    • Shortness of breath
    • Rapid heart beat

    After ruling out other causes, doctors diagnose the disorder with blood and urine tests.

    Treatment & Care

    Mild cases of autoimmune hemolytic anemia often require no treatment and resolve on their own. The most common treatments for the disorder include:

    • Treatment of underlying conditions
    • Corticosteroids such as prednisone to suppress the immune response
    • Immunosuppressive drugs such as rituximab or azathioprine
    • Splenectomy—to preserve RBC and prevent anemia
    • Blood transfusions 

    Long-term Outlook

    The prognosis for children with autoimmune hemolytic anemia is generally very good, and the anemia is typically short-lived and self-limiting. If it occurs in the first year of life or during teen years, the disease can often follow a chronic course that requires long-term immunosuppressive therapy, which can have serious consequences, such as increased risk of infection.