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

Hugo Benalcazar

Medically reviewed by Hugo Benalcazar, Northwestern University on December 24th, 2023.

Key Takeaways

  • The condition occurs when antibodies coat red blood cells, causing them to be destroyed in the spleen and liver at rates up to 10 times normal, leading to hemoglobin levels dropping below 10 g/dL.
  • Warm antibody type (85% of cases) causes chronic symptoms and responds well to steroids, while cold antibody type triggers acute episodes in cold temperatures and requires different treatment approaches.
  • The direct antiglobulin test (Coombs' test) detects antibodies on red blood cell surfaces with 95% accuracy, while elevated lactate dehydrogenase and low haptoglobin levels confirm active hemolysis.
  • First-line treatment involves prednisone at 1-2 mg/kg daily for 2-4 weeks, with 70-80% of patients achieving remission, though many require long-term immunosuppression with rituximab or splenectomy.
  • Secondary cases linked to chronic lymphocytic leukemia, systemic lupus erythematosus, or medications like quinidine require treating the underlying trigger for sustained improvement.

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.

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

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.

For more information on autoimmune hemolytic anemia, visit:

The Bottom Line

Early recognition and treatment of this immune-mediated destruction of red blood cells can prevent life-threatening complications and achieve remission in most patients. The key is distinguishing between warm and cold antibody types through specialized blood tests to guide appropriate therapy. If you're experiencing unexplained fatigue, jaundice, or dark urine, Doctronic can help connect you with specialists for rapid diagnosis and treatment.

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