Thrombotic thrombocytopenic purpura: symptoms and treatment (2023)

overview

What is thrombotic thrombotic thrombocytopenic purpura?

Thrombotic thrombocytopenic purpura (PTT) is a rare diseaseblood disease. With PTT, thrombi (blood clot) develop in small vessels throughout the body. These thrombi can restrict blood flow to vital organs in the body, including the heart, kidneys, and brain. Serious medical problems can occur as a result.

Because TTP causes increased clotting, more platelets are consumed. This can lead to internal bleeding, bleeding under the skin, and other bleeding problems.

Bleeding under the skin can cause small red or purple spots called petechiae.(Sport-__Tee_-kee-ay). These patches form on the skin and resemble a rash.

The term "thrombotic" refers to the thrombi or blood clots that develop. "thrombocytopenia' means that the number of platelets in the blood is reduced. "Purple" refers to purple spots and bruises on the skin.

What types of thrombotic thrombocytopenic purpura are there?

There are two main types of thrombotic thrombocytopenic purpura:inheritedmiacquired. Hereditary TTP is passed from parent to child due to variants in the ADAMTS13 gene. But most cases of PTT are acquired, meaning it develops later in life. Acquired TTP occurs when your body mistakenly produces antibodies that block the activity of the ADAMTS13 enzyme, which normally helps the blood to clot.

Who is affected by thrombotic thrombocytopenic purpura?

Thrombotic Thrombotic Thrombocytopenic purpura affects people of all ages, but most commonly occurs in people between the ages of 20 and 50. TTP is sometimes associated with pregnancy and collagen diseases (a group of diseases affecting connective tissue). It is also more common in people who have oneHIV.

How common is thrombotic thrombocytopenic purpura?

Thrombotic thrombocytopenic purpura is a rare condition, affecting approximately 4 in 100,000 people each year.

symptoms and causes

What are the most common symptoms of thrombotic thrombocytopenic purpura?

People with PTT can have several warning signs. Common symptoms of thrombotic thrombocytopenic purpura are:

• Bleeding on the skin or mucous membranes.
• Anämie.
• fatigue.
• Weakness.
• Confusion.
• Fever.
• AVK.
• Seizure.
• Headache.
• shortness of breath.
• Rapid heart rate (more than 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 is a lack of activity in the ADAMTS13 gene, blood becomes overactive, causing tiny blood clots to form in the body's small blood vessels. Clotting requires platelets. Because many of your blood platelets are used to form these tiny clots, not many are left to aid in clotting when the skin is cut. As a result, you may bleed for longer than normal.

What are the risk factors for thrombotic thrombocytopenic purpura?

Experts are not sure what factors trigger thrombotic thrombocytopenic purpura. However, the condition could potentially be related to:

• Krebs.
• The pregnancy.
• HIV.
• Lupus.
• stem cell orbone marrow transplants.
• Certain medications, such aschemotherapy,Ciclosporin AmiClopidogrel.

diagnosis and testing

How is thrombotic-thrombotic-thrombocytopenic purpura diagnosed?

Your doctor will perform a physical exam and ask about your symptoms and medical history. If your provider suspects TTP, he or she will order diagnostic testing.

What tests can confirm the diagnosis of thrombotic-thrombotic-thrombocytopenic purpura (TTP)?

Diagnostic tests to confirm thrombotic thrombocytopenic purpura may include:

• Complete blood count(CBC).A blood count measures thenumber of plateletsand the number of red and white blood cells. People with PTT have lower red blood cell and platelet counts.
• Bilirubin-Test.Hemoglobin (a protein) is released into the blood when red blood cells are damaged. The hemoglobin is then broken down into bilirubin (a compound). A blood test can determine the level of bilirubin in the blood. If your bilirubin levels are high, it could mean you have PTT.
• blood smears.Your doctor places a blood sample on a glass slide and examines it under a microscope. When you have TTP, your red blood cells will burst and rupture.
• Orina fromrenal function tests.These tests can tell your doctor if your kidneys are working properly. People with PTT may have blood cells or proteins in their urine.
• Kreatinin-Tests.Creatinine is a residue found in the blood. Normally, the kidneys remove creatinine from the body. If you have TTP, your creatinine levels may be high.
• Laktatdehydrogenase-Test.Lactate dehydrogenase (LDH) is an enzyme found in almost every cell in the body. When cells are destroyed, LDH is released into the blood. So if you have PTT, your LDH blood levels will be elevated.
• prueba ADAMTS13.This test measures the activity of the enzyme ADAMTS13. Lack of activity may indicate TTP.

management and treatment

How is thrombotic-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.

• Plasmaaustausch:This life-saving procedure removes your abnormal plasma and replaces it with healthy plasma. During the procedure, your doctor will place an IV line in your vein. Your blood is taken from your body and run through a centrifuge, which removes the plasma. The plasma is then added to your blood and returned to your body. Plasma exchange is used to treat acquired TTP. If plasma exchange is not possible, fresh frozen plasma can be used in the meantime.
• Fresh Frozen Plasma:Commonly used to treat hereditary TTP, fresh frozen plasma is administered via an intravenous (IV) line inserted into the arm. It replaces the missing or altered ADAMTS13 enzyme.
• Plasma therapy is completed in a hospital. Treatment continues until symptoms improve. Depending on your specific situation, this can take days or weeks.

medicine

Certain medications can slow or stop the formation of anti-ADAMTS13 antibodies. Common medications used to treat PTT include:rituximabeand glucocorticoids.

Operation

In severe cases, aSplenectomyit is necessary. The spleen produces the antibodies that block the ADAMTS13 enzyme, so removing the spleen can help solve this problem.

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

Recovery depends on several factors, including the severity of your condition and the treatment you receive. Most people receiving plasma therapy for TTP require several days to a few weeks to heal. Those who undergo a splenectomy generally take four to six weeks to recover.

prevention

Can I reduce my risk of thrombotic thrombocytopenic purpura?

Both types of PTT develop suddenly without a clear cause. As a result, avoiding it is not possible. However, if you've had PTT, you can talk to your doctor about ways to reduce your risk of flare-ups.

Outlook / Forecast

What can I expect if I have thrombotic-thrombotic-thrombocytopenic purpura?

Thrombotic thrombocytopenic purpura is a medical emergency. Once you've been diagnosed with the condition, your doctor will want to check your progress regularly.

How long does thrombotic thrombocytopenic purpura last?

In most cases, TTP occurs suddenly and lasts for days or weeks. However, it can take months in some cases. Shootings are also possible.

Is thrombotic thrombocytopenic purpura fatal?

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

Without treatment, PTT has a 90% mortality rate. However, with proper care, this rate drops to 10% to 20%.

to live with

When should I see my doctor?

You should make an appointment with your doctor any time you experience symptoms of TTP, such as: B. Tiredness, shortness of breath or purple spots on your skin. If you've already been diagnosed with TTP, watch for signs of a recurrence. In most cases, your doctor will restart plasma therapy to help bring your symptoms back under control.

What questions should I ask my doctor?

If you have PTT, ask your doctor about medicines that may increase your risk of bleeding. These include blood thinners like aspirin and ibuprofen.

If your child has PTT, be sure to ask your provider if certain activities should be restricted.

A note from the Cleveland Clinic

Thrombotic Thrombocytopenic Purpura is a serious condition that needs immediate treatment. Be sure to call your doctor or visit your local emergency room if you develop bothersome symptoms, such as purple spots on your skin. With the right treatment, most people with TTP can lead normal lives. However, keep in mind that your doctor may need to run regular tests to monitor your progress and reduce the risk of flare-ups.