is idiopathic autoimmune hemolytic anemia?
autoimmune hemolytic anemia is a form of autoimmune hemolytic anemia. Autoimmune
hemolytic anemia (AIHA) is a group of rare but serious blood disorders.
They occur when the body destroys red blood cells more rapidly than it produces
them. A condition is considered idiopathic when
its cause is unknown.
Autoimmune diseases attack
the body itself. Your immune system produces antibodies to help target foreign
invaders such as bacteria and viruses. In the case of autoimmune disorders,
your body mistakenly produces antibodies that attack the body itself. In AIHA,
your body develops antibodies that destroy red blood cells.
Idiopathic AIHA can be life-threatening because of its
sudden onset. It requires immediate medical attention and hospitalization.
is at risk?
About 50 percent of
all AIHA cases are idiopathic. AIHA can occur at any point in life and can
develop suddenly or gradually. It more commonly affects women.
If AIHA isn’t idiopathic, it’s because it was caused by an
underlying disease or medication. However, idiopathic AIHA has no obvious
causes. People with idiopathic AIHA may have only abnormal blood test results
and no symptoms.
of idiopathic AIHA
You may feel weak and short of breath if you develop sudden-onset
idiopathic AIHA. In other instances, the condition is chronic and develops over
time, so symptoms are less obvious. In both cases, symptoms may include one or
more of the following:
- increasing weakness
- shortness of breath
- rapid heartbeat
- pale or yellow-colored skin
- muscle pain
- dark-colored urine
- a headache
- abdominal discomfort
Your doctor will speak with you extensively about your
specific symptoms if they suspect you have AIHA. They’ll need to diagnose you
with AIHA and rule out medications or other underlying disorders as the
possible causes of the AIHA before diagnosing you with the idiopathic type.
First, your doctor will ask you about your medical history. It’s
likely they’ll admit you to the hospital for immediate testing and monitoring
if your symptoms are serious. Examples of serious issues include discolored
skin or urine or severe anemia. They may refer you to a blood specialist, or a hematologist.
You’ll need to have an extensive series of blood tests to
confirm AIHA. Some of the tests will measure the body’s red blood cell count. If
you have AIHA, your red blood cell count number will be low. Other tests will look
for certain substances in the blood. Blood tests that reveal an incorrect ratio
of immature to mature red blood cells may indicate AIHA. A high number of immature
red blood cells indicate that the body is trying to compensate for the mature
red blood cells that are being destroyed too quickly
Other blood test findings include a higher-than-normal level
of bilirubin and a decreased level of a protein called haptoglobin.
Bilirubin is a natural byproduct of red blood cell breakdown. These levels
become high when large numbers of red blood cells are destroyed. The
haptoglobin blood test can be especially useful in diagnosing AIHA. In
conjunction with other blood tests, it reveals that the protein is being
destroyed along with mature red blood cells.
In some cases, the typical lab results for these blood tests
may not be enough to diagnose AIHA, so your doctor may need to perform more
tests. Other tests, including the direct and indirect Coombs tests, can detect
increased antibodies in the blood. Urinalysis and a 24-hour urine collection
may reveal abnormalities in the urine, such as high levels of protein.
options for IAIHA
People suspected of having sudden onset idiopathic AIHA will
generally be hospitalized immediately because of its acute nature. Chronic
cases may often come and go without explanation. It’s possible for the
condition to improve without treatment.
Your doctor will monitor your blood glucose levels closely
if you have diabetes. Diabetes is a major risk factor for deaths from infection
as a result of treatment.
The first-line treatment is typically steroids such as
prednisone. They may help improve red blood cell counts. Your doctor will
carefully monitor you to check that the steroids are working. Once your condition
goes into remission, your doctor will try to wean you off of the steroids
slowly. People with AIHA undergoing steroid therapy may need supplements during
treatment. These could include:
- vitamin D
- folic acid
Your doctor may suggest surgical removal of the spleen if the
steroids don’t work completely. Removal of the spleen can reverse the
destruction of red blood cells. This surgery is known as a splenectomy. Two-thirds of
people who undergo a splenectomy have a partial or total remission from their
AIHA, and people with the idiopathic type tend to have the most successful
Other treatment options are immune-suppressing drugs, such
These can be effective medications for people who don’t successfully respond to
treatment with steroids or who aren’t candidates for surgery.
In some cases, the medication
rituximab may be preferred over the traditional immune-suppressing drugs. Rituximab
is an antibody that directly attacks specific proteins found on certain immune
It can be difficult to get a quick diagnosis of this
condition in cases where the cause of it is unknown. Treatment is sometimes delayed
in these cases. Idiopathic AIHA can be fatal if left untreated.
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.