What is idiopathic thrombocytopenic purpura (ITP)? ITP is a blood disorder characterized
by an abnormal decrease in the number of platelets in the blood. Platelets are
cells in the blood that help stop bleeding. A decrease in platelets can result
in easy bruising, bleeding gums, and internal bleeding.
- "Idiopathic" means the cause is unknown.
- "Thrombocytopenia" means a decreased number of platelets in the blood.
- "Purpura" refers to the purple discoloring of the skin, as with a bruise.
Who is
affected by ITP? ITP affects approximately 3,000 children under the age of 16 each year in
the US. There are two forms of ITP:
- acute thrombocytopenic purpura
This is most commonly seen in
young children (2 to 6 years old). The symptoms may follow a viral illness, such
as chickenpox. Acute ITP usually has a very sudden onset and the symptoms
usually disappear in less than six months (often within a few weeks). The
disorder usually does not recur. Acute ITP is the most common form of the
disorder.
- chronic thrombocytopenic purpura
The onset of the disorder can
happen at any age, and the symptoms can last a minimum of six months or several
years. Adults have this form more often than children, but it does affect
adolescents. Females have it two to three times more often than males. Chronic
ITP can recur often and requires continual follow up care with a blood
specialist (hematologist).
What causes ITP? In most cases, the cause of ITP is
unknown. It is not contagious, meaning a child cannot "catch it" from playing
with another child with ITP. It is also important to know that nothing the
parents, nor the child, did caused the disorder.
Often, the child may
have had a virus or viral infection about three weeks before developing ITP. It
is believed that the body, when making antibodies against a virus,
"accidentally" also made an antibody that can stick to the platelet cells. The
body recognizes any cells with antibodies as foreign cells and destroys them.
That is why ITP is also referred to as immune thrombocytopenic purpura.
The bone marrow is the soft, spongy center of the long bones and is
responsible for making blood cells, including platelets. The bone marrow
responds to the low number of platelets and produces many more to send out to
the body. A physician can look at the cells in the bone marrow and, in a child
with ITP, would see many young platelets that have been produced. However, the
blood test results of the circulating blood would show a very low number of
platelets. The body is producing the cells normally, but the body is also
destroying them. In most cases, other blood tests are normal except for the low
number of platelets. ITP platelets usually survive only a few hours, in
comparison to normal platelets which have a lifespan of seven to 10 days.
Platelets are essential for the formation of a blood clot. Blood clots
consist of a mass of fibers and blood cells. Platelets travel to a damaged area
and stick together to form a plug, whenever a person is cut, for example. If
there are not enough platelets, a clot cannot be formed, resulting in more
bleeding.
There has been research involved in looking at certain
medications causing ITP. Some medications may result in the altering of platelet
function. At this time, no direct link has been made with any specific
medication that may cause ITP.
What are the symptoms of
ITP? Normal
platelet count is in the range of 150,000 to 450,000. With ITP, the platelet
count is less than 100,000. By the time significant bleeding occurs, the child
may have a platelet count of less than 10,000. The lower the platelet count, the
greater the risk of bleeding.
Because platelets help stop bleeding, the
symptoms of ITP are related to increased bleeding. However, each child may
experience symptoms differently. Symptoms may include:
- purpura - the purple color of the skin after blood has "leaked" under it. A
bruise is blood under the skin. Children with ITP may have large bruises from no
known trauma. Bruises can appear at the joints of elbows and knees just from
movement.
- petechia - tiny red dots under the skin that are a result of very small
bleeds.
- nosebleeds
- bleeding in the mouth and/or in and around the gums
- blood in the vomit, urine, or stool
- bleeding in the head - this is the most dangerous symptom of ITP. Any head
trauma that occurs when there are not enough platelets to stop the bleeding can
be life threatening.
The symptoms of ITP may resemble other
blood disorders or medical problems. Always consult your child's physician for a
diagnosis.
How
is ITP diagnosed? In addition to a complete medical history and physical examination,
diagnostic procedures for ITP may include the following:
- complete blood count (CBC) - a measurement of size, number, and
maturity of different blood cells in a specific volume of blood (to measure
platelets).
- additional blood and urine tests (measure bleeding time and detect
possible infections)
- careful review of the child's medications
Sometimes, a bone marrow aspiration is
performed to look at the production of platelets and to rule out any abnormal
cells the marrow may be producing that could lower platelet counts.
Treatment for
ITP: Specific
treatment for ITP will be determined by your child's physician based on:
- your child's age, overall health, and medical history
- type of disorder (acute versus chronic)
- severity and extent of the disorder
- your child's tolerance for specific medications, procedures, or therapies
- expectations for the course of the disease
- your opinion or preference
Not all children with ITP require
treatment. Close monitoring of your child's platelets and prevention of serious
bleeding complications may be the course of action chosen until the body is able
to correct the disorder on its own. Many children with ITP are able to
spontaneously recover in about two to four days.
When treatment is
necessary, the two most common forms of treatment are steroids and intravenous
gamma globulin:
- steroids
Steroids help prevent bleeding by decreasing the rate of
platelet destruction. Steroids, if effective, will result in an increase in
platelet counts seen within two to three weeks. Side effects may include
irritability, stomach irritation, weight gain, hypertension, and acne.
- intravenous gamma globulin (IVGG)
Intravenous gamma globulin
(IVGG) is a protein that contains many antibodies and also slows the destruction
of platelets. IVGG works more quickly than steroids (within 24 to 48 hours).
Other
treatments for ITP may include:
- Rh immune globulin
This medication temporarily stops the spleen
from destroying platelets. Your child must be Rh positive and have a spleen for
this medication to be effective.
- medication changes
If it is a medication that is the suspected
cause, discontinuation or changing the medication may be necessary.
- infection treatment
If infection is the cause for ITP, then
treatment of the infection may result in higher platelet counts.
- splenectomy
In some cases, the child's spleen may need to be
removed since this is the site of platelet destruction. This is considered more
often in older children with chronic ITP to decrease the rate of platelet
destruction.
- hormone therapy
Teenage girls may need to take hormones to stop
their menstrual cycle when their platelets are low if excessive bleeding occurs.
Preventing bleeding: The parents of a child with the
disorder need to be aware of how to prevent injuries and bleeding. Consider the
following:
- For the young child, make the environment as safe as possible. Padding a
crib, wearing helmets, and providing protective clothing are necessary when
platelet counts are low.
- Contact sports, riding bicycles, and rough play may need to be restricted.
- Avoid medications which contain aspirin, as they may interfere with the
body's ability to control bleeding.
It is important to discuss with your
child's physician other limitations necessary to prevent injuries in a child
with ITP.
Long-term outlook for a child with ITP: Although there is no known cause of ITP
and there is no cure, the prognosis for a child with ITP is very good.
Usually, the body stops making the antibodies that are attacking the
platelets and the disorder resolves on its own. The goal of treatment is to keep
the child's platelets in a safe range until the body corrects the problem.
Overall, prevention of serious bleeding, such as head injuries, is the
most significant factor in prognosis. Providing a safe environment, prompt
medical attention, and continued medical care are all indications for a long
term, healthy prognosis.
Page forms part of www.apls.tk, the information site on ANTIPHOSPHOLIPID SYNDROME (APS or ANTIPHOSPHOLIPID SYNDROME (APLS))
Medical Keywords: systemic antiphospholipid antibody syndrome, Antiphospholipid, Antiphospholipid Antibody Syndrome, Antiphospholipid Syndrome, APS, APLS, Hughes
Syndrome, Sticky Blood, Clotting Disorder, Stroke, TIA, PE, death, Antiphospholipid Antibody Syndrome, Antiphospholipid Syndrome, APS, APLS,
Hughes Syndrome, Sticky Blood, Clotting Disorder, Stroke, TIA, PE, death
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