Immune Thrombocytopenia

What is immune thrombocytopenia (ITP)?

Immune thrombocytopenia (ITP) is a form of low platelet count, a condition that keeps your blood from clotting. When your blood can’t make clots, you may bruise easily, bleed a lot when you’re hurt or start bleeding for no reason. Immune thrombocytopenia happens when your immune system clears your platelets from circulation and your platelet level goes down. In some cases, ITP goes away on its own or with treatment. Other times, immune thrombocytopenia is a chronic condition, which means symptoms can be treated, but not cured. People with chronic ITP may need treatment for the rest of their lives.

What are other names for ITP?

• Autoimmune thrombocytopenic purpura.
• Immune thrombocytopenic purpura.
• Idiopathic thrombocytopenic purpura.
• Werlhof disease.
• Autoimmune thrombocytopenia.

How common is immune thrombocytopenia?

It’s rare. Each year, about 4 in 100,000 children and 3 in 100,000 adults in the United States learn they have this condition.

Types of immune thrombocytopenia

The two ITP types are:

• Primary ITP: This is when your immune system attacks your platelets. About 80% of all cases are primary ITP. Healthcare providers may call immune thrombocytopenia an autoimmune disorder.
• Secondary ITP: This may happen if you have underlying conditions like chronic infections, blood cancers or autoimmune disorders that affect your platelet levels.

Healthcare providers classify ITP by the amount of time you’ve had the condition:

• Acute ITP, which usually goes away within three months. Acute immune thrombocytopenia affects more children than adults.
• Persistent ITP, which lasts between three and 12 months.
• Chronic ITP, which lasts a year or more.

What are ITP symptoms?

You may not have any symptoms, but they can happen slowly or quickly if you do. Symptoms may include:

• Petechiae: This symptom appears as tiny red or purple dots on your lower legs that resemble a rash.
• Purpura: This is when petechiae join together, forming red, purple or brown spots on your skin. The spots are larger than petechiae but smaller than a bruise. This happens when small blood vessels under your skin leak blood.
• Bruises: Bruises happen when blood pools under your skin. You may notice you’re developing bruises more easily than usual or have bruises you can’t explain.
• Bleeding gums: You may notice blood on your toothbrush and your gums may appear swollen.
• Blood in poop (stool): Your poop may appear very dark.
• Blood in urine (pee): If toilet water is pale pink after you pee, you may have blood in your urine.
• Heavy menstrual periods: If your periods last longer than seven days or you’re bleeding more than usual, you may have menorrhagia.
• Heavy nosebleeds.
• Hematoma (large bruise): This is when a lot of blood accumulates in one spot under your skin.
• Fatigue.

What causes immune thrombocytopenia?

Immune thrombocytopenia happens when your immune system makes antibodies that mistakenly identify your cells as being invaders, and then direct other immune cells to attack your platelets. When something damages or cuts your blood vessels, platelets swarm the damaged area, sticking together to make a blood clot that stops bleeding (primary hemostasis). Experts don’t know what triggers the immune system attacks on platelets.

Research shows people with human immunodeficiency virus (HIV), H. pylori infections or hepatitis C have an increased risk of developing ITP.

How is immune thrombocytopenia diagnosed?

Before making a diagnosis, your healthcare provider will do a physical exam to check for bleeding on or under your skin. They’ll ask about your medical history. That’s because ITP symptoms are like other bleeding disorder symptoms. Likewise, ITP may be a complication of blood cancer. Your provider will eliminate other possible causes before making a diagnosis.

What tests diagnose immune thrombocytopenia?

Providers may do the following tests:

• Complete blood count (CBC).
• Peripheral blood smear.

If you’re at risk for HIV, hepatitis C or H. pylori, your provider may do tests for those infections.

What’s the treatment for immune thrombocytopenia?

Often, children with immune thrombocytopenia have mild symptoms and don’t need treatment, but most adults will. If you do need treatment, your healthcare provider may prescribe medications to boost your platelet count or keep your immune system from attacking your platelets. Medications may include:

• Corticosteroids to temporarily block the antibodies that destroy platelets.
• Immunoglobulin or thrombopoietin receptor agonists to boost your platelet production.
• Immunosuppressants to suppress your immune system.

Your provider may recommend other steps like:

• Treating underlying infection: If an infection is temporarily making your platelet level drop, getting rid of the infection can help with bleeding issues.
• Avoiding certain medications: Your provider may ask you to quit taking aspirin or another medicine that can make you more likely to bleed.
• Having a platelet transfusion: If your bleeding is serious, you may need to receive platelets that another person has donated to a blood bank.
• Having surgery: In some cases, your provider may decide to remove your spleen through surgery. This may help your immune thrombocytopenia, but it can also put you more at risk for infections.

Treatment side effects

Medications, transfusions and surgery may cause several different side effects. Your healthcare provider will discuss treatment side effects, so you know what to expect.

What’s the outlook for immune thrombocytopenia?

The outlook varies depending on your or your child’s unique situation. If your child has ITP, they’ll probably get better within a few weeks or months without needing treatment. If you have persistent or chronic ITP, you may need medications and other treatments to manage your symptoms depending on how low your platelet count is, but you’ll always have the condition. Healthcare providers can’t cure immune thrombocytopenia, but many people with chronic ITP live for decades with the condition.

How do I take care of myself?

If you have ITP, you may need medication for the rest of your life to ensure your body makes enough platelets. And you’ll need to avoid activities that increase the chance you’ll get hurt and start bleeding. For example, you should:

• Avoid contact sports like football, basketball and ice hockey where you might get hit or knocked down.
• If your child has ITP, they should wear protective helmets and gear any time they do activities like riding bicycles or skateboards.
• Remember to wear your seatbelt when driving or riding in a vehicle, and be sure your child wears their seatbelt.
• Talk to your provider about over-the-counter medication or herbal supplements, so you can avoid supplements or medication that could make you more likely to bleed.

When should I see my healthcare provider?

Contact your provider if you notice new bruises or petechia that may be signs that your condition is getting worse. Your provider may do tests to check your platelet levels.

When should I go to the emergency room?

Very rarely, immune thrombocytopenia may make your platelet levels drop so low that you start bleeding uncontrollably. Any bleeding that you can’t stop by applying pressure is a medical emergency and a need to go to the emergency room.

What questions should I ask my healthcare provider?

• What type of ITP do I have?
• What treatment do you recommend?
• How long will the treatment take to work?
• Will I always need treatment?
• What if the first treatment doesn’t work?

What happens if I have ITP and get pregnant?

It may help to know that your fetus won’t be affected by ITP. Your baby may have fewer platelets at birth, but their platelet count will increase. If you have a mild case of immune thrombocytopenia during pregnancy, you won’t need treatment. If you do need treatment during pregnancy, your provider will recommend treatments that won’t affect your fetus.