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Understanding Autoimmune Hemolytic Anemia (AIHA): Causes, Symptoms, Diagnosis, and Treatment

Autoimmune hemolytic anemia (AIHA) is a rare type of anemia where your body's immune system mistakenly attacks and destroys red blood cells faster than your bone marrow can make new ones. Red blood cells are essential for carrying oxygen throughout your body. When you have too few red blood cells, your body can't get enough oxygen, leaving you feeling tired or short of breath.

Types of AIHA

AIHA can be classified in a few different ways:

  • Primary AIHA: Appears by itself

  • Secondary AIHA: Affects you because you have another illness

  • Warm antibody hemolytic anemia: The immune reaction takes place at or above normal body temperature

  • Cold antibody hemolytic anemia: Red blood cells are destroyed when you're exposed to cold

Causes of AIHA

You can get AIHA if you have an autoimmune disease like lupus. Other diseases and medications can also cause AIHA, including:

  • Cancers, such as chronic lymphocytic leukemia and non-Hodgkin's lymphoma

  • Infections like Mycoplasma pneumoniae

  • Medicines such as penicillin, methyldopa (Aldomet), quinine (Qualaquin), and sulfonamides

  • Viruses such as Epstein-Barr virus, cytomegalovirus, HIV, and hepatitis

Symptoms of AIHA

Many of the symptoms of AIHA are similar to other forms of anemia, including:

  • Chills

  • Fast heartbeat (tachycardia)

  • Pale skin that may start to yellow

  • Shortness of breath

  • Weakness and fatigue

  • Chest pain

  • Yellow skin or whites of the eyes (jaundice)

  • Dark urine

  • A feeling of abdominal fullness related to an enlarged spleen

With cold antibody hemolytic anemia, you may also notice cold hands and feet, a bluish or reddish color in hands and feet, headache, digestive problems, and pain in the back and legs.

Diagnosing AIHA

If you think you could have any type of anemia, talk to your doctor. They may refer you to a hematologist, a doctor who specializes in blood diseases. Your doctor will discuss your medical history, medications, and symptoms, and request a blood test called a complete blood count (CBC) to look for signs of anemia.

If your CBC test results point to anemia, your doctor might want to do more tests, such as:

  • Reticulocyte count

  • Coombs' test

  • Peripheral smear

  • Bilirubin test

  • Haptoglobin test

  • Cold agglutinin titer

Treating AIHA

Treatment for AIHA depends on the underlying cause and severity of the condition. If you have a disease like lupus that's causing your anemia, your doctor will treat it first. If a medication is the cause, you'll likely have to stop taking that medicine.

If your AIHA is mild, you may not need treatment. Doctors usually first prescribe steroids, such as hydrocortisone or prednisone, to stop your immune system from attacking your red blood cells. A medicine called rituximab may make steroids work even better.

If you don't improve, you may need surgery to remove your spleen, where much of the destruction of red blood cells takes place. Other medicines such as azathioprine (Imuran) and cyclophosphamide (Cytoxan) can be used to suppress the immune system. In some cases, you may need a blood transfusion.

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