Is Idiopathic Autoimmune Hemolytic Anemia?
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:
- 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.
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:
or yellow-colored skin
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
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).
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.