Thrombotic Thrombocytopenic Purpura

What is thrombotic thrombocytopenic purpura?

Thrombotic thrombocytopenic purpura (TTP) is a rare blood disorder. With TTP, thrombi (blood clots) develop in small vessels throughout your body. These thrombi can restrict the flow of blood to your body’s vital organs, including the heart, kidneys and brain. As a result, serious medical problems can arise.

Because TTP causes increased clotting, more blood platelets are used up. This can cause internal bleeding, bleeding underneath the skin and other bleeding problems.

Bleeding under the skin can cause tiny red or purple dots called petechiae (peh-\_tee\-kee-ay). These dots form on your skin and resemble a rash.

The term “thrombotic” refers to the thrombi — or blood clots — that develop. “Thrombocytopenic” means that there are a reduced number of platelets in the blood. “Purpura” refers to the purplish dots and bruises on the skin.

What are the types of thrombotic thrombocytopenic purpura?

There are two main types of thrombotic thrombocytopenic purpura: inherited and acquired. Inherited TTP is passed down from parents to children due to variants in the ADAMTS13 gene. But most cases of TTP are acquired, which means it develops later in life. Acquired TTP happens when your body mistakenly makes antibodies that block activity from the ADAMTS13 enzyme, which normally supports blood clotting function.

Who does thrombotic thrombocytopenic purpura affect?

Thrombotic thrombocytopenic purpura affects people of all ages, but it mostly occurs in people aged 20 to 50. TTP is sometimes associated with pregnancy and collagen-vascular disease (a group of disorders that affect connective tissue). It also occurs more frequently in people who have HIV.

How common is thrombotic thrombocytopenic purpura?

Thrombotic thrombocytopenic purpura is a rare disease, occurring in approximately 4 out of 100,000 people each year.

What are common thrombotic thrombocytopenic purpura symptoms?

People with TTP may experience a number of warning signs. Common thrombotic thrombocytopenic purpura symptoms include:

• Bleeding into the skin or mucus membranes.
• Anemia.
• Tiredness.
• Weakness.
• Confusion.
• Fever.
• Stroke.
• Seizure.
• Headaches.
• Shortness of breath.
• Fast heart rate (over 100 beats per minute).
• Jaundice (yellowing of the skin).

What causes thrombotic thrombocytopenic purpura?

The ADAMTS13 enzyme is a protein in the blood that helps with clotting. When there’s a lack of activity in the ADAMTS13 gene, it leads to overactive blood clotting, causing small clots to form in the small vessels throughout your body. Clotting requires platelets. Because so many of your platelets are being used to form these small clots, there aren’t many left over to help with clotting when you cut your skin. As a result, you may bleed longer than normal.

What are thrombotic thrombocytopenic purpura risk factors?

Experts aren’t exactly sure which factors trigger thrombotic thrombocytopenic purpura. However, the condition could potentially be linked to:

• Cancer.
• Pregnancy.
• HIV.
• Lupus.
• Stem cell or bone marrow transplants.
• Certain medications, such as chemotherapy, cyclosporine A and clopidogrel.

How is thrombotic thrombocytopenic purpura diagnosed?

Your healthcare provider will perform a physical examination and ask about your symptoms and health history. If your provider suspects TTP, they will order diagnostic tests.

Which tests can confirm a diagnosis of thrombotic thrombocytopenic purpura (TTP)?

Diagnostic tests used to confirm thrombotic thrombocytopenic purpura may include:

• Complete blood count (CBC). A CBC measures the number of platelets as well as the number of red blood cells and white blood cells. People with TTP have a lower number of red blood cells and platelets.
• Bilirubin test. Hemoglobin (a protein) is released into your blood when red blood cells are damaged. Hemoglobin is then broken down into bilirubin (a compound). A blood sample can determine the bilirubin levels in your blood. If your bilirubin levels are high, it could mean that you have TTP.
• Blood smear. Your provider places a sample of your blood on a glass slide and examines it under a microscope. If you have TTP, your red blood cells will be broken and torn.
• Urine or kidney function tests. These assessments can tell your healthcare provider if your kidneys are functioning properly. People with TTP may have blood cells or protein in their urine.
• Creatinine test. Creatinine is a waste product found in the blood. Typically, the kidneys remove creatinine from the body. If you have TTP, your creatinine levels may be high.
• Lactate dehydrogenase test. Lactate dehydrogenase (LDH) is an enzyme that’s found in nearly every cell in your body. When cells are destroyed, LDH is released into your blood. So, if you have TTP, the LDH levels in your blood will be elevated.
• ADAMTS13 assay. This test measures the activity of the ADAMTS13 enzyme. If there’s a lack of activity, it could indicate TTP.

How is thrombotic thrombocytopenic purpura treated?

In most cases, plasma therapy is used to treat TTP. Other options include medication and surgery.

Plasma treatments

There are two main types of plasma therapy for TTP: plasma exchange and fresh frozen plasma infusion.

• Plasma exchange: This lifesaving procedure removes your abnormal plasma and replaces it with healthy plasma. During the procedure, your healthcare provider places an IV line in your vein. Your blood is taken from your body and passed through a centrifuge, which removes the plasma. Next, plasma is added to your blood and put back into your body. Plasma exchange is used to treat acquired TTP. If plasma exchange isn’t available, then fresh frozen plasma may be used in the meantime.
• Fresh frozen plasma: Typically used to treat inherited TTP, fresh frozen plasma is given through an intravenous (IV) line that’s inserted into your arm. It replaces the missing or altered ADAMTS13 enzyme.
• Plasma therapy is completed in a hospital. Treatments continue until your symptoms improve. Depending on your unique situation, this can take days or weeks.

Medication

Certain medications can slow or halt anti-ADAMTS13 antibodies from forming. Common medications used to treat TTP include rituximab and glucocorticoids.

Surgery

In severe cases, a splenectomy is necessary. The spleen creates the antibodies that block the ADAMTS13 enzyme, so removing the spleen can help address this problem.

How long does it take to recover from thrombotic thrombocytopenic purpura treatment?

Recovery depends on several factors, including the severity of your condition and the treatment that you receive. Most people who have plasma therapy for TTP will need several days up to a few weeks of healing time. Those who have a splenectomy usually need about four to six weeks to recover.

Can I reduce my risk for thrombotic thrombocytopenic purpura?

Both types of TTP develop suddenly with no clear cause. As a result, it’s not possible to prevent it. However, if you’ve had TTP, you can talk to your healthcare provider about ways to reduce your risk of flare-ups.

What can I expect if I have thrombotic thrombocytopenic purpura?

Thrombotic thrombocytopenic purpura is a medical emergency. If you’ve been diagnosed with the condition, your healthcare provider will want to monitor your progress periodically.

How long does thrombotic thrombocytopenic purpura last?

In most cases, TTP comes on suddenly and lasts for days or weeks. However, it can last for months in some instances. Flare-ups are also possible.

Is thrombotic thrombocytopenic purpura fatal?

Without prompt treatment, TTP can be fatal or cause long-lasting health problems such as stroke or brain damage. Therefore, if you show any signs or symptoms, make an appointment with your healthcare provider right away.

Without treatment, TTP has a 90% mortality rate. However, with proper care, that rate is reduced to 10% to 20%.

When should I see my healthcare provider?

You should schedule a visit with your healthcare provider anytime you notice TTP symptoms, such as fatigue, shortness of breath or purplish spots on your skin. If you’ve already been diagnosed with TTP, watch out for relapse symptoms. In most cases, your provider will restart plasma therapy to get your symptoms under control again.

What questions should I ask my healthcare provider?

If you have TTP, ask your healthcare provider about medications that could increase your bleeding risk. These include blood thinners, such as aspirin and ibuprofen.

If your child has TTP, be sure to ask your provider whether certain activities should be limited.