Idiopathic Thrombocytopenic Purpura (ITP)

ITP Overview

ITP is an autoimmune disorder that causes the body to destroy its platelets. People with ITP develop bruising, bleeding into the skin, and often bleeding from mucous membranes such as the nose or mouth. A very rare but serious complication of ITP is bleeding into the head. This is usually caused by some type of trauma to the head.

Idiopathic means occurring for no known cause

Thrombocytopenia means a decreased number of platelets in the blood

Purpura refers to the purple discoloring of the skin, similar to a bruise

The Bone marrow is the soft, spongy center of the bone and is home to the stem cells that are 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. In a child with ITP, many young platelets are present, but tests of circulating blood show very low numbers of platelets. The body is producing the cells normally, but it is also destroying them. In most cases other blood tests are normal. In children with ITP, platelets only survive a few hours, instead of the normal 7 to 10 days.

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: Most commonly seen in children between the ages 2 to 6 years old. The symptoms may follow a viral illness, such as chickenpox. The most common form, Acute ITP usually has a very sudden onset and the symptoms usually last between a few weeks to 6 months, and rarely reoccur. With treatment, the risk of serious of life threatening bleeding is very low.

Chronic Thrombocytopenic Purpura: This disorder can occur at any age, and the symptoms last a minimum of 6 months and can persist for many years. Chronic ITP is most common in adults, rarely affecting children and adolescents. Females are two or three times more likely to have the disease than males. Chronic ITP can become a lifelong problem and regular treatment with a hematologist (blood specialist) is recommended.

ITP is also found in many women prior to and during their pregnancy. 

Women diagnosed with ITP prior to conception:

There is no research that suggests women with ITP should not become pregnant, yet physicians will explain that maternal and fetal complications may occur. As a result careful monitoring by physicians is necessary and it is suggested that women with ITP have an obstetrician, hematologist, and pediatrician/neonatologist work closely together.

Women both with and without ITP can expect a fall in their platelet count during pregnancy, especially during the third trimester. Because of this it is recommended that women with ITP have their platelet count carefully monitored throughout the pregnancy. Women with ITP should not take many of the medicines used to treat ITP during pregnancy and should talk to their doctor if they are taking medicine and wish to become pregnant

Women who learned they have ITP during their pregnancy

It is difficult to diagnose ITP during pregnancy because platelet counts can be low for many other reasons. A doctor may not be able to make a diagnosis of ITP during pregnancy but when there are not any other causes found to explain a low platelet count, the diagnosis is often ITP.

ITP's affect on the fetus and newborn

During pregnancy the mother's antibodies may cross the placental barrier and upon recognizing the fetal platelets, lead to their destruction. Pregnant women with ITP can therefore induce moderate to severe thrombocytopenia in the fetus or in the newborn. However, the overall risk of long term complications in the newborn is low. In most cases thrombocytopenia in newborns can be treated without any lasting effects. While a newborn's thrombocytopenia peaks 3 to 5 days following the delivery, the platelet count recovers without lasting consequence within 6 to 8 weeks. Women who have already given birth to a thrombocytopenic child can expect subsequent pregnancies to result in an infant with thrombocytopenia.

What causes ITP?

The main cause of ITP is still unknown. The illness is not contagious, meaning a child cannot catch it from being in contact with a child with ITP. It must be known that nothing the child or parents ever did was the cause for this disorder.

In ITP, the body produces antibodies that stick to the platelet cells. Normal antibodies are proteins that guard against infection. The spleen, an organ that is responsible for removing disease causing cells from the body, recognizes the antibodies as signs of foreign cells and destroys the platelets to which they are attached.

Often, a child with ITP may have a viral infection a few weeks before developing evidence of ITP. Viral infections may trigger ITP. While making antibodies to the virus, the body may make abnormal antibodies that lead to platelet destruction.

Researchers have explored the possibility that certain medications cause ITP. While we know that some medications can alter platelet function, no direct link has been made to any specific medication and ITP.

Symptoms 

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 will cause a greater risk of bleeding.

Because the 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 bones, 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 gums

Blood in urine, stool, or vomit

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.

How ITP is Diagnosed?

ITP is diagnosed by taking the patients history, performing a complete medical exam, and evaluating blood tests. Diagnostic procedures include:

Complete blood count (CBC): A measurement of size, number, and maturity of different blood cells (including platelets) in a specific volume of blood.

Additional blood and urine tests ( to measure clotting times and detect possible infections)

Possible bone marrow testing to identify any abnormal cells

Careful review of the child's medications

Treatment

A number of treatments can help increase platelet levels in people with ITP, but there is no cure for the disorder. Majority of children get better spontaneously, with or without treatment in a matter of days to weeks.

Common forms of treatment are:

Steroids

Intravenous Gamma Globulin

Rh Immune Globulin

Preventing Bleeding

Parents and caretakers of a child with ITP need to be aware of how to prevent injuries and bleeding, and should consider the following:

Make the environment as safe as possible for young children. Padding a crib, wearing helmets, and providing protective clothing are necessary when platelet counts are low. 

Certain sports and rough play need to be closely monitored or restricted.

Avoid medications that may interfere with platelet function and may cause bleeding. These include Aspirin and non-steroidal, anti-inflammatory medications (aspirin, ibuprofen, motrin, Advil, naproxen, naprosyn, Aleve).

Long Term Outlooks

Although there is no known cause for ITP there is also no cure. However, the prognosis for a child with ITP is very good. 

Usually, the body stops making the antibodies that attack the platelets and the disorder resolves on its own. The goal of treatment is to keep the child safe and prevent bleeding 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.

Facts about ITP

ITP occurs in around 2 to 5 of every 100,000 children.

ITP is most common in children around the age of 5 or 6, but it can occur at any age

Contact a CCBF doctor:

If you have questions about ITP or want to schedule an appointment, please call 212-746-3400 to schedule a visit with Dr. Bussel.

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