Autoimmune Hemolytic Anemia: Symptoms, Causes & Treatment

Autoimmune hemolytic anemia (AIHA) is a type of anemia in which abnormal antibodies attack red blood cells. This causes the cells to break down too early and can lead to symptoms like fatigue, paleness, dizziness, and yellowing of the skin and eyes.

In many cases, the exact cause cannot be identified. AIHA may develop after an infection, with autoimmune diseases such as lupus or rheumatoid arthritis, with the use of certain medications, or in the setting of cancer.

If autoimmune hemolytic anemia (AIHA) is suspected, it’s important to see a primary care provider or a hematologist to confirm the diagnosis. Although there is no cure, AIHA can often be managed effectively with medications such as corticosteroids or other immunosuppressants, and in some cases, surgery.

doctor explaining results to patient

Common symptoms

The main symptoms of autoimmune hemolytic anemia are:

  • Weakness, fatigue, or feeling unwell;

  • Paleness or dizziness;

  • Sleepiness;

  • Fast heartbeat;

  • Shortness of breath;

  • Abdominal swelling;

  • Yellowing of the skin and eyes;

  • Dark, cola-colored urine.

These symptoms are similar to those of other types of anemia. For this reason, if AIHA is suspected, it is important to see a hematologist or a primary care provider to confirm the diagnosis and begin the most appropriate treatment.

Confirming a diagnosis

Autoimmune hemolytic anemia is diagnosed by a hematologist or a primary care provider through a review of symptoms, health history, physical exam, and blood tests.

Main tests

To confirm the diagnosis of autoimmune hemolytic anemia, the doctor may order tests such as:

  • Complete blood count (CBC), to confirm anemia and assess its severity;

  • Immunologic tests, such as the direct Coombs test, which checks for antibodies attached to the surface of red blood cells;

  • Blood reticulocyte count, which shows how the body is trying to compensate for red blood cell destruction and is usually elevated in hemolytic anemia;

  • Total and fractionated bilirubin, which may be abnormal, especially indirect (unconjugated) bilirubin;

  • Peripheral blood smear, which can reveal specific changes in red blood cells;

  • LDH (lactate dehydrogenase), which often increases when red blood cells are being destroyed.

A healthcare provider may also order other blood tests, such as iron, vitamin B12, and ferritin levels. These tests help distinguish autoimmune hemolytic anemia from other types of anemia.

Possible causes

Autoimmune hemolytic anemia is caused by the production of abnormal antibodies that attack red blood cells. This leads to their destruction, which is called hemolysis.

This can occur with conditions such as lupus, rheumatoid arthritis, lymphoma, and leukemia. It may also be linked to the use of certain medications, such as some antibiotics, although sometimes no specific cause is found.

AIHA can also develop after infections, including those caused by Epstein-Barr virus or Parvovirus B19. It may also occur after bacterial infections such as Mycoplasma pneumoniae or Treponema pallidum.

Warm vs cold

Autoimmune hemolytic anemia can be classified as warm or cold, depending on the type of antibody involved in attacking red blood cells. 

According to the American Society of Hematology, warm autoimmune hemolytic anemia is typically driven by IgG autoantibodies, while cold agglutinin disease is caused by IgM autoantibodies.

Treatment options

Treatment of autoimmune hemolytic anemia should be guided by a hematologist. It may include medications such as corticosteroids, immunosuppressants, and immunomodulators.

In more severe cases, blood transfusions may be recommended to help control the anemia.

Surgery to remove the spleen, known as splenectomy, may sometimes be performed. This is more likely when other treatments have not been effective.

When the underlying cause of AIHA is identified, treating it whenever possible is also important for helping control the anemia.

Can autoimmune hemolytic anemia be cured?

Autoimmune hemolytic anemia has no cure. However, red blood cell destruction can often be controlled with appropriate treatment.

Even with proper treatment, the anemia can sometimes return. This is more likely if the anemia was severe at the start of the illness.