Immune thrombocytopenia

Synonyms

2

Overview

Immune thrombocytopenia is a bleeding disorder also known as Idiopathic thrombocytopenia purpura (ITP) and affects children and adults. Children often develop ITP after a viral infection and usually recover fully without treatment. In adults, the disorder is often long term.

It is a disorder that can lead to easy or excessive bruising and bleeding. The bleeding results from unusually low levels of platelets — the cells that help blood clot.

In most cases, an autoimmune response is thought to cause Immune thrombocytopenia. Normally, your immune system helps your body fight off infections and diseases. But if you have Immune thrombocytopenia, your immune system attacks and destroys its own platelets. The reason why this happens isn't known.

Symptoms

Immune thrombocytopenia (ITP) may not cause any signs or symptoms. However, Immune thrombocytopenia can cause bleeding inside the body (internal bleeding) or underneath or from the skin (external bleeding). Signs of bleeding may include:

• Bruising or purplish areas on the skin or mucous membranes (such as in the mouth). These bruises are called purpura. They're caused by bleeding under the skin, and they may occur for no known reason.
• Pinpoint red spots on the skin called petechiae. These spots often are found in groups and may look like a rash. Bleeding under the skin causes petechiae.
• A collection of clotted or partially clotted blood under the skin that looks or feels like a lump. This is called a hematoma.
• Nosebleeds or bleeding from the gums (for example, during dental work).
• Blood in the urine or stool (bowel movement).

Any kind of bleeding that's hard to stop could be a sign of ITP. This includes menstrual bleeding that's heavier than normal. Bleeding in the brain is rare, and its symptoms may vary.

A low platelet count doesn't directly cause pain, problems concentrating, or other symptoms. However, a low platelet count might be associated with fatigue (tiredness).

Causes

In most cases, an autoimmune response is thought to cause immune thrombocytopenia (ITP).

Normally, your immune system helps your body fight off infections and diseases. In Immune thrombocytopenia, however, your immune system attacks and destroys your body's platelets by mistake. Why this happens isn't known.

In some people, Immune thrombocytopenia may be linked to viral or bacterial infections, such as HIV, hepatitis C, or H. pylori.

Children who have acute (short-term) Immune thrombocytopenia often have had recent viral infections. These infections may "trigger" or set off the immune reaction that leads to Immune thrombocytopenia.

Prevention

You can't prevent immune thrombocytopenia (ITP), but you can prevent its complications.

• Talk with your doctor about which medicines are safe for you. Your doctor may advise you to avoid medicines that can affect your platelets and increase your risk of bleeding. Examples of such medicines include aspirin and ibuprofen.
• Protect yourself from injuries that can cause bruising or bleeding.
• Seek treatment right away if you develop any infections. Report any symptoms of infection, such as a fever, to your doctor. This is very important for people who have Immune thrombocytopenia and have had their spleens removed.

Diagnosis

Tests usually include:

• A complete blood count. This test checks the number of red blood cells, white blood cells, and platelets in your blood. In ITP, the red and white blood cell counts are normal, but the platelet count is low.
• A blood smear. For this test, some of your blood is put on a slide. A microscope is used to look at your platelets and other blood cells.

You also may have a blood test to check for the antibodies (proteins) that attack platelets.

If blood tests show that your platelet count is low, your doctor may recommend more tests to confirm a diagnosis of ITP. For example, bone marrow tests can show whether your bone marrow is making enough platelets.

If you're at risk for HIV, hepatitis C, or H. pylori, your doctor may screen you for these infections, which might be linked to ITP.

Some people who have mild ITP have few or no signs of bleeding. They may be diagnosed only if a blood test done for another reason shows that they have low platelet counts.

Prognosis

Immune thrombocytopenia (ITP) is a fairly common blood disorder. Both children and adults can develop ITP. Children usually have the acute (short-term) type of ITP. Acute ITP often develops after a viral infection. Adults tend to have the chronic (long-lasting) type of ITP. Women are two to three times more likely than men to develop chronic ITP.

Treatment

Treatment for immune thrombocytopenia (ITP) is based on how much and how often you're bleeding and your platelet count.

Adults who have mild Immune thrombocytopenia may not need any treatment, other than watching their symptoms and platelet counts. Adults who have Immune thrombocytopenia with very low platelet counts or bleeding problems often are treated.

Children who have mild Immune thrombocytopenia may not need treatment other than monitoring and followup to make sure their platelet counts return to normal.

Medicines

Medicines often are used as the first course of treatment for both children and adults.

Corticosteroids (cor-ti-co-STEER-roids), such as prednisone, are commonly used to treat Immune thrombocytopenia. However, steroids have many side effects. Some people relapse (get worse) when treatment ends.

Other medicines also are used to raise the platelet count such as rituximab, immune globulin, and anti-Rh (D) immunoglobulin.

Medicines also may be used with a procedure to remove the spleen called splenectomy (splee-NECK-tuh-mee).

If medicines or splenectomy don't help, two newer medicines—eltrombopag and romiplostim—can be used to treat Immune thrombocytopenia.

Removal of the Spleen (Splenectomy)

If needed, doctors can surgically remove the spleen. This organ is located in the upper left abdomen. The spleen is about the size of a golf ball in children and a baseball in adults.

The spleen makes antibodies (proteins) that help fight infections. In Immune thrombocytopenia, these antibodies destroy platelets by mistake.

If Immune thrombocytopenia hasn't responded to medicines, removing the spleen will reduce the destruction of platelets. However, it also may raise your risk for infections. Before you have the surgery, your doctor may give you vaccines to help prevent infections.

If your spleen is removed, your doctor will explain what steps you can take to help avoid infections and what symptoms to watch for.

Other Treatments

Platelet Transfusions

Some people who have Immune thrombocytopenia with severe bleeding may need to have platelet transfusions and be hospitalized. Some people will need platelet transfusions before having surgery.

For a platelet transfusion, donor platelets from a blood bank are injected into the recipient's bloodstream. This increases the platelet count for a short time.

For more information about platelet transfusions, go to the Health Topics Blood Transfusion article.

Treating Infections

Some infections can briefly lower your platelet count. Treating the infection may help increase your platelet count and reduce bleeding problems.

Stopping Medicines

Some medicines can lower your platelet count or cause bleeding. Stopping the medicine can sometimes help raise your platelet count or prevent bleeding.

For example, aspirin and ibuprofen are common medicines that increase the risk of bleeding. If you have Immune thrombocytopenia, your doctor may suggest that you avoid these medicines. 

If you have immune thrombocytopenia (ITP), you can take alternative steps to prevent complications. Lifestyle changes and ongoing care can help you manage the condition.

Lifestyle Changes

Try to avoid injuries, especially head injuries, that can cause bleeding in the brain. For example, don't take part in contact sports, such as boxing, football, or karate. Other sports, such as skiing or horseback riding, also put you at risk for injuries that can cause bleeding. However, some safe activities like swimming, biking (with a helmet), and walking are ok. Ask your doctor about physical activities that are safe for you.

Take precautions such as regular use of seatbelts and wearing gloves while working with knives and other tools.

If your child has ITP, ask his or her doctor whether you need to restrict your child's activities.

Ongoing Care

You may want to find a doctor who is familiar with treating people who have ITP. For example, hematologists are doctors who specialize in diagnosing and treating blood disorders. Discuss with your doctor how to manage ITP and when to seek medical care.

Talk with your doctor before taking prescription medicines or over-the-counter medicines, supplements, vitamins, or herbal remedies. Some medicines and supplements can affect platelets and increase your chance of bleeding. Common examples are aspirin or ibuprofen. Your doctor may advise you to avoid these medicines.

Watch for symptoms of infection, such as a fever, and report them to your doctor promptly. If you've had your spleen removed, you may be more likely to become ill from certain infections.

Immune Thrombocytopenia in Pregnancy

In women who are pregnant and have ITP, the ITP usually doesn't affect the baby. However, some babies may be born with or develop low platelet counts soon after birth.

The babies' platelet counts almost always return to normal without any treatment. Treatment can speed up recovery in the babies whose platelet counts are very low.

Treatment for ITP during pregnancy depends on a woman's platelet count. If treatment is needed, the doctor will take a close look at the possible effects of the treatment on the unborn baby.

Women who have mild cases of ITP usually can go through pregnancy without treatment. Pregnant women who have very low platelet counts or a lot of bleeding are more likely to have heavy bleeding during delivery or afterward. To prevent heavy bleeding, these women usually are treated.