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What is autoimmune hemolytic anemia?

Autoimmune hemolytic anemia (AIHA) is a rare red blood cell disorder that occurs when antibodies directed against a person’s own red blood cells cause them to burst, leading to insufficient concentration in the blood. When this happens, the lifetime of red blood cells decrease from 100 to 120 days to just a few days in serious cases. Intracellular components of red blood cells 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, lupus, 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 | Symptoms & Causes

What are the symptoms of autoimmune hemolytic anemia?

The most common symptoms of autoimmune hemolytic anemia (AIHA) are:

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

What causes AIHA?

In approximately half of cases the cause of autoimmune hemolytic anemia, whether idiopathic or primary, cannot be determined. This condition can also be caused by or occur in tandem with other, or secondary, disorders. It can also rarely occur following the use of certain drugs, like penicillin, or after a person has a blood and marrow stem cell transplant.

Is autoimmune hemolytic anemia inherited?

Researchers believe that there are multiple possible factors involved in AHA's causes — including genetic and environmental influences. In a very small number of cases, autoimmune hemolytic anemia appears to run in families. In those cases, the gene appears recessive

Autoimmune Hemolytic Anemia | Diagnosis & Treatments

How is autoimmune hemolytic anemia diagnosed?

After ruling out other causes, doctors diagnose autoimmune hemolytic anemia (AIHA) with blood and urine tests. It is important to understand that some symptoms of anemia may resemble those of other more common medical problems or other blood disorders. Because some of these symptoms can also point to other conditions, and because anemia itself can be a symptom of another medical problem, it’s important to have your child evaluated by a qualified medical professional for an accurate diagnosis and prompt treatment.

How is autoimmune hemolytic anemia treated?

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

What is the long-term outlook for children with AIHA?

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.

How we care for AIHA

Children with autoimmune hemolytic anemia receive treatment at Dana-Farber/Boston Children’s Cancer and Blood Disorders Center through our Rare Anemias and Iron Disorders Program. Our patients have access to the broadest set of blood disorder expertise and pediatric subspecialties available.

The breadth of our expertise allows us to assemble a team of specialists to meet the specific needs of the patient. Our treatment teams are led by a pediatric hematologist who specializes in inherited iron deficiency disorders, as well as nurse practitioners and physician assistants who specialize in treating anemias, hemoglobinopathies, and red blood cell disorders.

Autoimmune Hemolytic Anemia | Programs & Services