TTP disease is a life-threatening blood clotting disorder that causes blood and platelet-rich blood clots to clog the smallest blood vessels, especially the brain and kidneys, causing severe organ damage. You can find more information below.

What is TTP disease?

TTP disease, also known as thrombotic thrombocytopenic purpura , is a rare serious blood disorder. “Thrombotic” refers to blood clots that form. “Thrombocytopenic” means that the blood is less rich in platelets. “Purpura” refers to purple areas caused by bleeding under the skin. Therefore, the long name of TTP disease is thrombotic thrombocytopenic purpura.

Major symptoms include a drastic decrease in the number of blood platelets, abnormal destruction of red blood cells ( hemolytic anemia ), and disorders of the nervous system and other organs as a result of their tiny clots forming in the smallest arteries.

What causes TTP disease?

Lack of activity in the ADAMTS13 enzyme (a type of protein in the blood) causes thrombotic thrombocytopenic purpura. The ADAMTS13 gene controls the enzyme involved in blood clotting.

Not having enough enzyme activity causes overactive blood clotting. In TTP disease, blood clots form in small blood vessels in the body. These clots can restrict or block the flow of oxygen-rich blood to organs of the body, such as the brain, kidneys, and heart. As a result, serious health problems can develop.

The increased clotting that occurs in TTP disease also depletes most of the platelets in the blood. Bleeding problems can occur when there are fewer platelets in the blood. People with TTP may bleed inside their body, under the skin, or from the surface of the skin. When cut or injured, they may bleed longer than usual.

TTP disease can also cause red blood cells to break down before new ones can form. This leads to hemolytic anemia.

Hereditary causes of TTP

In hereditary TTP, the ADAMTS13 gene is defective. It does not stimulate the body to make a normal ADAMTS13 enzyme. As a result, enzyme activity is missing or altered.

“Inherited” means the condition is passed from parents to children through genes. A person who inherits TTP is born with two copies of the faulty gene, one from each parent.

Often, each parent has one copy of the faulty gene but no signs or symptoms of TTP.

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Causes of acquired TTP

In acquired TTP, the ADAMTS13 gene is not defective. Instead, the body makes antibodies (proteins) that block the activity of the ADAMTS13 enzyme.

“Acquired” means you were not born with this condition, but you develop it some time after birth.

Who is at risk?

It is known which triggers cause TTP disease, but some factors may play a role. These factors can include:

  • Pregnancy, cancer, HIV , lupine s and certain diseases and conditions such as infections
  • Certain medical procedures, such as surgery, blood and stem cell transplants
  • Certain medications, such as chemotherapy, hormone therapy, and estrogens
  • Quinine, an ingredient often found in tonic water and nutritional supplements

What are the symptoms of TTP disease?

Blood clots, low platelet count, and damaged red blood cells cause the signs and symptoms of thrombotic thrombocytopenic purpura (TTP).

Signs and symptoms include:

  • Bruise-like purplish areas on the skin or mucous membranes. These things called purpura are caused by bleeding under the skin.
  • Spot-sized red or purple spots on the skin. These spots, called petechiae , are often found in groups and may look like a rash. Bleeding under the skin causes petechiae.
  • Paleness or jaundice (a yellowish color of the skin or the whites of the eyes)
  • Fatigue (feeling very tired and weak)
  • Fire
  • rapid heart rate or shortness of breath
  • Headache, speech changes, confusion, paralysis, or seizure
  • blood in the urine

When should you see a doctor?

If you have TTP and have any of these signs or symptoms, you may be having a relapse (exacerbation). The best thing to do in such a situation is to see a doctor.

How is TTP disease diagnosed?

Your doctor will diagnose thrombotic thrombocytopenic purpura (TTP) based on your medical history, physical exam, and test results. If TTP disease is suspected or diagnosed, a hematologist will be involved in your treatment. A hematologist is a doctor who specializes in the diagnosis and treatment of blood disorders.

medical history

Your doctor will ask about factors that can affect TTP. For example, they may want to learn about:

  • certain conditions, such as cancer, HIV, lupus, infections, or whether you are pregnant
  • Prior medical procedures such as blood and bone marrow stem cell transplant
  • whether you are taking ticlopidine, clopidogrel, hormone therapy, estrogens, or chemotherapy
  • Whether you use products containing quinine

physical examination

As part of the medical history and physical exam, your doctor will ask about the symptoms you have and look for signs such as :

  • Bruising and bleeding under your skin
  • Fire
  • Paleness or jaundice (a yellowish color of the skin or the whites of the eyes)
  • fast heart rate
  • Awareness changes, such as speech changes or confusion
  • Changes in urine (such as blood in the urine)
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Diagnostic tests

Your doctor may also recommend the following tests to find out if you have TTP:

  • complete blood count

For TTP disease, this test measures the number of red blood cells, white blood cells, and platelets in your blood. For this test, a blood sample is usually taken from a vein in your arm. If you have TTP, you have fewer platelets and red blood cells than normal.

  • blood smear test

For this test, a blood sample is usually taken from a vein in your arm. Some of your blood is placed on a glass slide. A microscope is then used to look at your red blood cells. In TTP, red blood cells rupture and break.

  • Platelet count

This test counts the number of platelets in a blood smear. People with TTP have fewer platelets than normal in their blood. This test is used with a blood smear to help diagnose TTP.

  • Bilirubin tests

When red blood cells die, they release a protein called hemoglobin into the bloodstream . The body breaks down hemoglobin into a compound called bilirubin. High bilirubin in the bloodstream causes jaundice. For this blood test, a blood sample is usually taken from a vein in your arm. The bilirubin level in the sample is checked. If you have TTP, your bilirubin level may be high because your body is breaking down red blood cells faster than normal.

  • Kidney function and urine tests

These tests show whether your kidneys are working well. If you have TTP, your urine may contain protein or blood cells. You may also have high blood creatinine levels. Creatinine is a blood product that is normally removed by the kidneys.

  • Coombs lyrics

This blood test is used to find out if hemolytic anemia is the cause of TTP. For this test, a blood sample is usually taken from a vein in your arm. Hemolytic anemia occurs in TTP because red blood cells break down as they try to squeeze out blood clots. When TTP is caused by hemolytic anemia, the Coombs test is negative. The test is positive if the antibodies destroy the red blood cells.

  • Lactate dehydrojenaz testi

This blood test measures a protein called lactate dehydrogenase (LDH). For this test, a blood sample is usually taken from a vein in your arm. Hemolytic anemia causes red blood cells to break down and LDH to be released into the blood. LDH is also released from tissues injured by blood clots as a result of TTP.

  • ADAMTS13 testi

Lack of activity in the ADAMTS13 enzyme causes TTP. For this test, a blood sample is usually taken from a vein in your arm. The blood is sent to a special laboratory to test the enzyme’s activity.

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How is TTP disease treated?

Thrombotic thrombocytopenic purpura (TTP) can be fatal or cause permanent damage, such as brain damage or stroke, if not treated immediately.

In most cases, TTP comes on suddenly and lasts for days or weeks, but it can also last for months. Relapses (exacerbations) may occur in 60 percent of people who have had TTP. Exacerbations also occur in most people who inherit TTP.

Plasma therapies are the most common method of treating TTP. Other treatments include medications and surgery. Treatments are done in the hospital.

Plasma therapy

Plasma is the liquid part of your blood. It carries blood cells, hormones, enzymes, and nutrients throughout your body.

TTP disease is treated with plasma therapy as follows:

  • Fresh frozen plasma for people who have inherited TTP disease
  • Plasma exchange for patients with acquired TTP

Plasma therapy begins in the hospital when TTP is diagnosed or suspected.

For hereditary TTP, fresh frozen plasma is given through an intravenous (IV) line inserted into a vein. This is done to replace the missing or altered ADAMTS13 enzyme.

Plasma exchange ( also known as plasmapheresis ) is used to treat acquired TTP . This is a life-saving procedure. ADAMTS13 removes antibodies that damage your enzyme from the blood. Plasma exchange also replaces the ADAMTS13 enzyme.

An IV needle or tube is inserted into a vein in your arm to collect the blood during a plasma exchange. The blood passes through a cell separator that removes the plasma from the blood. The non-plasma portion of the blood is recorded and the donated plasma is added.

The blood is then put back to you through an IV line placed in one of your blood vessels. The time required to complete the procedure varies, but usually takes about 2 hours.

Fresh frozen plasma or plasma exchange treatments are usually continued until your blood test results and symptoms improve. It can take days or weeks depending on your situation. You will likely stay in the hospital during this time.

Some people who recover from TTP have flare-ups. This can happen in the hospital or after you go home. If you have a flare-up, your doctor will restart plasma therapy.

Other treatments

Other treatments are used if plasma therapy does not work well or if exacerbations occur frequently.

For acquired TTP disease, medications can slow or stop the formation of antibodies to the ADAMTS13 enzyme. Medications used to treat TTP include glucocorticoids, vincristine, rituximab, and cyclosporine A. However, your doctor will decide which medicine to take and how.

Sometimes surgery is needed to remove the spleen. This is because cells in the spleen produce antibodies that block ADAMTS13 enzyme activity.

On a small note , this disease should not be confused with ITP disease .

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