Thrombotic thrombocytopenia purpura (TTP) is a very rare and sometimes life-threatening blood disorder.

“Thrombotic” means that you tend to get blood clots. They form inside your body’s small blood vessels. The clots block blood flow to your heart, brain, and other organs.

“Thrombocytopenia” means that you have a low level of platelets in your blood. Platelets are important because they form clots to help you stop bleeding when you are injured. When you have TTP, your platelets are being used up by the many blood clots that your body forms. This means that if you do start bleeding from an injury, you might not have enough platelets left in your blood to clot and stop bleeding.

“Purpura” means that your skin bruises easily. When you have too few platelets in your blood, you may get bleeding under your skin. This may cause red or purple bruises or what look like small spots on your skin, which are the common symptoms of TTP.

TTP occurs mainly in women, especially during pregnancy. It can also be found in people who have conditions such as cancer, lupus, or HIV infection. Sometimes TTP can be inherited, though this is much rarer. Pregnancy can be a trigger for symptoms if you were born with TTP.

There are two types of TTP. They have different causes:

• Immune-mediated TTP (iTTP) is caused by a mistaken immune response in your body. Your immune system attacks a certain protein called ADAMTS13. When this happens, your blood platelets clot too much, even without an injury. This is the most common type of TTP, making up 95% of cases.
• Congenital TTP (cTTP) is rare, making up just 5% of all cases. It's also called inherited TTP or Upshaw-Shulman syndrome. This type of TTP is caused by a change in a gene. That means that you're born with this disorder. If you have cTTP, your body doesn’t make enough of the protein ADAMTS13. This leads to too many blood clots. It also causes low platelet counts in your blood.

Blood clots that form from TTP may affect different areas of your body. Symptoms can include:

• Belly pain.
• Blood in your urine.
• Bruising on your skin.
• Changes in your speech.
• Extreme tiredness.
• A fever.
• A low amount of urine.
• Headaches.
• Memory changes and confusion.
• Nausea or vomiting, or both.
• Red or purple spots (purpura).
• Seizures.
• Shortness of breath, pale skin, and a rapid heart rate.
• Very heavy menstrual flow.
• Weakness.
• A yellow tint to your skin or eyes.

To find out if you have TTP, your doctor will:

• Ask about your family history.
• Do a physical exam.
• Take a sample of your blood to see how well your platelets, red blood cells, and kidneys are working.
• Study your blood cells under a microscope. This will help your care team learn the quantity, shape, and size of your different cells.
• Find out your ADAMTS13 protein level count.

Your doctor may recommend the following treatments:

• Plasma exchange. Plasma is a clear, pale liquid that makes up just over half of your blood. One of its jobs is to help clean your blood of waste. You may get a plasma donation from someone with healthy plasma. This gives you more ADAMTS13 protein that you need to prevent blood clots. For this treatment, some of your blood is taken from you. Then your plasma is removed from it. Your blood is “cleaned up,” mixed with fresh donor plasma, and returned to your body.
• Plasma infusion. If you have congenital TTP, you may only need a plasma infusion instead of a full exchange. A newer medicine called recombinant ADAMTS13 is also now approved for people with congenital TTP.
• Immunotherapy. You may also receive a monoclonal antibody infusion (called rituximab). It works by reducing the number of antibodies in your blood, allowing your ADAMTS13 to rise.
• Corticosteroids. Your doctor will likely give you steroid medicine along with plasma exchange. This medicine helps calm the immune system so it stops attacking ADAMTS13.
• Caplacizumab. This is a newer medicine that helps stop blood clots from forming quickly. It's used along with plasma exchange and steroids. It can help your platelet count return to normal faster.

You can help yourself feel better and live well with some self-care at home.

• Apply firm pressure or ice to any area of bleeding for at least 5 minutes. Put a thin cloth between the ice and your skin.
• Be careful not to fall or bruise yourself by bumping into objects.
• Be careful when using sharp objects such as scissors.
• Don't blow your nose too hard or scratch the inside of your nose.
• Don't push too hard while having bowel movements.
• Don't use enemas, suppositories, or tampons.
• Don't walk barefoot. Wear slip-resistant footwear.
• Floss once each day if you usually floss. Don't floss areas that are sore or bleeding.
• Brush gently with a soft toothbrush.
• Talk to your doctor about what exercises are safe for you.
• Use stool softeners or laxatives if needed.

It’s important to contact your doctor right away or seek emergency treatment if you have:

• Any form of bleeding, including increased skin bruising, bleeding in your urine or bowel movements, or heavy menstrual bleeding.
• A rash that looks like tiny red spots.
• Belly pain, nausea, or vomiting.
• Confusion, numbness or weakness on one side of your body; seizures; speech changes; or visual disturbances of any type.
• Dark urine.
• A yellow tint to your skin or eyes.

TTP symptoms may return in some people who’ve had one episode of symptoms, sometime many years later. That’s why it’s important to stay in touch with your hematologist to watch you for any sign of a TTP relapse.