Immune Thrombocytopenia (ITP)

What is immune thrombocytopenia?

Immune thrombocytopenia (ITP) is a bleeding disorder that affects the blood’s ability to clot because of a low number of blood cells called platelets (thrombocytes).

Normally, when blood vessels are damaged from cuts or other injuries, platelets stick together to clot or seal the cut. This process is called coagulation—when blood cells pool together to stop the bleeding. Without enough platelets, a person with ITP can have bleeding under the skin or from injury and blood vessel damage.

A person with ITP often may have purple bruises (purpura) caused by bleeding from small blood vessels under the skin. This bruising appears on the skin or mucus membranes, such as inside the mouth. ITP can also cause bleeding that appears as pinpoint-sized red or purple dots on the skin (petechiae). These tiny dots many look like a skin rash.

With ITP, a person may also have nosebleeds, bleeding from the gums or other hard-to-stop bleeding. Women who have ITP may have heavier-than-normal menstrual bleeding.

Excessive bleeding can cause a hematoma—a pool of clotted blood under the skin that looks or feels like a lump. Bleeding in the brain caused by ITP is extremely rare but can be life threatening if it occurs.

The cause of ITP is thought to be an abnormal immune response. Normally, the immune system works to help the body fight off infection and disease. With ITP, the immune system attacks and destroys its own platelets for reasons that are not clearly understood.

There are two main types of ITP:

• Short-term (acute)—the most common type of ITP, which mainly affects children. Acute ITP often occurs after a viral infection and usually lasts less than a year.
• Long-term (chronic)—mostly occurs in adults, but some children and teens are affected. ITP lasting one year or longer is considered chronic and affects women two to three times more often than men.

There is no way of knowing who will have acute or chronic ITP. ITP is not contagious or infectious, meaning it cannot be passed on to another person.

How common is immune thrombocytopenia?

ITP is a relatively common blood disorder that can develop in both children and adults. About four to five of every 100,000 children are diagnosed with ITP each year. Routine blood tests can help make the diagnosis.

What are the signs and symptoms of immune thrombocytopenia?

Although ITP may not cause any signs or symptoms, it can cause bleeding inside the body, beneath the skin, or externally from a cut or other injury. Any bleeding that is hard to stop may be a sign of ITP.

Signs and symptoms of bleeding caused by ITP may include the following:

• Bruising or purplish areas on the skin or inside the mouth
• Tiny reddish spots on the skin that may look like a rash
• A lump under the skin caused by collected blood clots
• Nosebleeds
• Bleeding from the gums while brushing teeth or after dental work
• Blood in urine or stool
• Feeling overly tired

How is immune thrombocytopenia treated?

Treatment for ITP is based on how severe the bleeding is. In mild cases of ITP, no treatment may be needed.

ITP that occurs in children often goes away within a few weeks or months, even without treatment. Most will go away within one year of diagnosis. Children who have ITP with signs and symptoms of bleeding are often treated.

Teens or adults with chronic ITP also may not need treatment other than monitoring platelet counts and signs and symptoms of bleeding. But treatment may be needed if a person’s platelet count is very low or if bleeding problems are significant.

Current treatments for ITP include the following:

• Medication—is often the first-line treatment for ITP in both children and adults.
  
  • Corticosteroids are oral medicines that help increase platelet counts. These drugs have many side effects, and ITP can get worse for some people after they stop taking a corticosteroid. Prednisone is the corticosteroid drug commonly used to treat ITP.
  • Intravenous (IV) drugs used to help increase platelet counts in people with ITP include rituximab, immune globin and anti-Rh (D) immunoglobulin.
  • Some newer drugs, called eltrombopag and romplostim, can help the body make more platelets. The long-term effects of these drugs in children are still being studied.
• Removal of the spleen (splenectomy)—might be used to reduce how the immune system destroys platelets.
  • With ITP, antibodies produced by the spleen that help fight infection and disease also destroy platelets by mistake.
  • If ITP doesn’t respond to first-line medicines, removing the spleen through surgery can help protect platelets by stopping the destructive antibodies that the spleen produces. However, this procedure may raise the risk of infection.
• Platelet transfusion—is used to increase the platelet count on a short-term basis.
  • A person with ITP who has an episode of severe bleeding may require a platelet transfusion. In some cases, a platelet transfusion might be needed before surgery to control bleeding.
  • Donor platelets from a blood bank are injected into the bloodstream of the person with ITP.

What are the survival rates for immune thrombocytopenia?

For most children and adults, ITP is not serious or life-threatening.

In 80 percent of children with ITP, platelet counts return to normal within six to 12 months. Acute ITP in children usually lessens on its own within a few weeks or months and does not come back. No treatment may be needed. However, for a small number of children, ITP continues and may require treatment with medicines or surgery.

In adults, chronic ITP is different from person to person and can last for years. However, even a person with a severe form of chronic ITP can have a long life. Most people with chronic ITP are eventually able to stop treatment and maintain a safe platelet count.

Why choose St. Jude for your child’s immune thrombocytopenia treatment?

• St. Jude investigators take part in the ITP Consortium of North America (ICON). This group is focused on understanding the best treatment options for children with ITP and improving quality of life for affected children and their parents.
• St. Jude investigators regularly take part in clinical trials for new ITP therapies.
• The nurse-to-patient ratio at St. Jude is unmatched—averaging 1:3 in hematology and oncology, and 1:1 in the Intensive Care Unit.
• The  major treatment programs at St. Jude are so successful because the doctors and scientists work closely together to rapidly introduce promising therapies to the clinical setting.