Idiopathic Autoimmune Hemolytic Anemia

Written by Joan Jovinelly | Published on October 3, 2012
Medically Reviewed by George Krucik, MD

What Is Idiopathic Autoimmune Hemolytic Anemia?

Autoimmune hemolytic anemia (AIHA) is a group of rare but serious blood disorders. They occur when the body’s red blood cells are destroyed more rapidly than they are being produced. The condition is considered idiopathic when its cause is unknown.

In instances when the cause of AIHA is known, is it typically inherited or develops as a result of:

  • cancer
  • viral infection
  • another autoimmune disorder, such as lupus

AIHA may develop as a side effect of certain drugs in very rare cases. These drugs include antibiotics and over-the-counter nonsteroidal anti-inflammatory drugs (NSAIDs). Examples of NSAIDs include aspirin, ibuprofen, and naproxen (Polsdorfer, 2012).

AIHA developed from an overdose of lecithin in one extremely unusual case. In this case, the patient took a large number of lecithin supplements—over 5 capsules per day. Lecithin is a nutritional supplement that is typically harmless when taken in recommended doses (Cases Journal, 2009). It is used extensively in commercially prepared food items as a substitution for dairy protein. 

Autoimmune diseases attack the body itself. The body develops auto-antibodies that destroy red blood cells in the case of AIHA. The idiopathic form of the disorder is extremely serious. It is often life threatening because of its sudden onset. Idiopathic autoimmune hemolytic anemia (IAIHA) requires immediate medical attention and hospitalization.

Who Is at Risk?

About half of all cases of AIHA are caused by unknown reasons. The disorder is rare. It affects about one in every 100,000 people. It more commonly affects women. It can occur at any point in life and can develop suddenly or gradually (Blood, 2010). Idiopathic forms of the disorder are likely to have no obvious causes and patients may present with only abnormal blood test results and no symptoms. 

Symptoms of Idiopathic AIHA

A person may feel weak and short of breath when he or she develops AIHA. In other instances, the condition is chronic and develops over time so symptoms are less obvious. In either instance, symptoms may include any or all of the following:

  • increasing weakness
  • shortness of breath
  • rapid heartbeat
  • pale or yellow-colored skin
  • muscle pain
  • nausea and/or vomiting
  • dark-colored urine
  • headache
  • abdominal discomfort
  • feeling bloated
  • diarrhea

Diagnosing Idiopathic AIHA

Your doctor will speak with you extensively about your specific symptoms if he or she suspects AIHA. He or she will take a medical history and may refer you to a blood specialist (hematologist). You will likely be admitted to a hospital for immediate testing and monitoring if your symptoms are serious. Examples of serious issues include discolored skin and/or urine.

You’ll need to undergo an extensive series of blood tests to confirm AIHA. Some tests help identify the body’s red blood cell count. Other tests look for certain substances in the blood. Blood tests that reveal an incorrect ratio of mature to immature red blood cells may indicate AIHA. This is the body’s way of compensating for the mature red blood cells that are being destroyed. 

Other blood findings include a higher-than-normal level of bilirubin and a decreased level of a protein called haptoglobin (Hp or HPT). Bilirubin is a substance found in bile that is a byproduct of destroyed red blood cells. The haptoglobin blood test is especially useful in diagnosing AIHA. In conjunction with other blood tests, it reveals that protein is being destroyed in the bloodstream along with mature red blood cells (Lab Tests Online, 2012). 

Other decisive tests like the direct/indirect Coombs’ test can detect increased antibodies in the blood. Additionally, urinalysis and/or a 24-hour urine collection may reveal abnormalities in the urine. Urine tests are useful for detecting high levels of protein.       

Treatment Options for IAIHA

People suspected of having sudden onset IAIHA will generally be hospitalized immediately because of its acute nature. 

Chronic cases may often come and go without explanation. It is therefore possible for the condition to improve on its own. 

The first-line treatment is steroids such as prednisone. They may help improve red blood cell counts. Your doctor may suggest surgical removal of the spleen if steroids fail to work completely. Removal of the spleen can reverse the destruction of red blood cells. Immune-suppressing drugs are a third option. These can be an effective treatment for patients who do not successfully respond to treatment with steroids or surgery (NIH, 2010).

Long-Term Outlook

Idiopathic AIHA in children is typically short-lived. The condition is often chronic in adults, and can flare up or reverse itself without explanation. AIHA is highly treatable in both adults and children. Most people make a full recovery.

Swift diagnosis often presents a challenge in instances where the cause of the condition is unknown. Treatment is sometimes delayed in these cases. AIHA can be fatal if left untreated. 

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