Thrombotic thrombocytopenic purpura (TTP) is a rare blood disorder in which thrombi (blood clots) form in small blood vessels throughout your body. These blood clots can restrict the flow of oxygen-rich blood to your organs, causing a number of life-limiting complications. Treatments include plasma therapy, medication and surgery.
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.
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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.
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.
Thrombotic thrombocytopenic purpura is a rare disease, occurring in approximately 4 out of 100,000 people each year.
People with TTP may experience a number of warning signs. Common thrombotic thrombocytopenic purpura symptoms include:
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.
Experts aren’t exactly sure which factors trigger thrombotic thrombocytopenic purpura. However, the condition could potentially be linked to:
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.
Diagnostic tests used to confirm thrombotic thrombocytopenic purpura may include:
In most cases, plasma therapy is used to treat TTP. Other options include medication and surgery.
There are two main types of plasma therapy for TTP: plasma exchange and fresh frozen plasma infusion.
Certain medications can slow or halt anti-ADAMTS13 antibodies from forming. Common medications used to treat TTP include rituximab and glucocorticoids.
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.
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.
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.
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.
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.
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%.
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.
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.
A note from Cleveland Clinic
Thrombotic thrombocytopenic purpura is a serious condition that must be treated immediately. Be sure to call your healthcare provider or visit your local emergency room if you develop troublesome symptoms, including purplish spots on your skin. With proper treatment, most people with TTP can lead normal lives. Keep in mind, however, that your healthcare provider may need to run tests periodically to monitor your progress and reduce your risk for flare-ups.
Last reviewed by a Cleveland Clinic medical professional on 02/09/2022.
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