Thrombotic Thrombocytopenic Purpura (TTP)

Overview of Thrombotic Thrombocytopenic Purpura (TTP)

Thrombotic thrombocytopenic purpura (TTP) is a rare blood condition that leads to the formation of blood clots in vessels. Blood clotting also known as thrombosis occurs in smaller-sized blood vessels.

Formation of clots can hinder the normal blood flow across these vessels leading to dysfunction of organs i.e. kidney, brain, heart. TTP can significantly reduce your red blood cells and platelet count. A lower platelet count can lead to uncontrollable bleeding when injured. 

People who suffer from TTP can bleed under the skin layer and inside the body. This underneath bleeding appears on the skin as purple-colored spots, known as purpura. 

Other than purpura, small red colored dots can also appear on skin that is known as petechiae and can appear like a rash. TTP also leads to the rapid destruction of red blood cells causing hemolytic anemia.

Signs and Symptoms of Thrombotic Thrombocytopenic Purpura (TTP)

Some of the common symptoms of TTP include: 

  • Influenza-like illness (early signs)
  • Fatigue
  • Confusion
  • Seizures
  • Headaches
  • Shortness of breath
  • Paleness
  • Faster heartbeat
  • Fever
  • Mental state changes
  • Poor kidney function
  • Uncontrollable bleeding

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Causes of Thrombotic Thrombocytopenic Purpura (TTP)

The problem of TTP is caused by the formation of clots that end up blocking blood vessels across the body.  

Mechanism behind TTP:

TTP can occur because of the inhibitory action of an antibody against an enzyme known as ADAMTS13. This is a protease enzyme that affects a protein breakdown i.e., vWF (von Willebrand factor). Under normal circumstances, the presence of larger units of these proteins causes the formation of blood clots.  

The larger subunits are destroyed by the activity of enzymes. In the case of TTP, when the enzyme ADAMTS13 isn’t sufficiently produced and can’t break down the vWF protein. This causes the formation of blood clots that occur in TTP. Destruction of the ADAMTS13 enzyme can occur in two ways:

  1. Autoimmune: In this case, the body produces such antibodies that destroy the ADAMTS13 enzyme stopping the breakdown of vWT.

  2. Genetic: In this case, genetic changes are the key factor. Changes in the ADAMTS13 gene occur and pass on to the next generation. This genetic mutation works through a similar mechanism and results in clotting. TTP caused by this condition is known as Upshaw-Schulman syndrome. 

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Risk Factors of Thrombotic Thrombocytopenic Purpura (TTP)

The condition usually arises from the breakdown of ADAMTS13 enzyme that is responsible for coagulation. Though autoimmune response and genetic factors are mainly responsible, certain factors can increase the risk of suffering from TTP.

Gene: Genetics is an important risk factor for the disease because children can inherit the faulty gene from their parents. 

Medical Conditions: Suffering from certain medical conditions such as HIV, cancer, infections, lupus, etc. can increase your chances of suffering from TTP.

Medical Procedures: Medical procedures such as blood and bone marrow transplants can increase your chances of developing the disease.

Medicines: Certain medications serve as an important risk factor for TTP such as clopidogrel, hormonal therapy, quinine, chemotherapy medicines, cyclosporine A etc.

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Prevention

Both types of TTP can begin suddenly without showing any clear signs and symptoms. So, you can’t prevent the disease in both cases.

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Diagnosis

The problem of TTP can begin anytime without any clear warning or sign. Diagnosis of TTP can consist of the following steps:

Physical Examination: Your physician can begin with a physical examination once you have signs and symptoms. A part of this examination involves the inquiry regarding your family history etc.

Blood Tests: To further confirm the condition, your physician can ask you to go for certain diagnostic procedures. These procedures include: 

  • CBC: Complete blood count test is one of the most common procedures performed to diagnose the presence of platelets in your blood sample. Your blood profile reveals the shape, size, and count of platelets in your blood.
  • Platelet Count: Numbering your platelets can further help your physician to look at things more closely.
  • Blood Smear: A blood smear test is used to reveal the morphology of red blood cells that get damaged in TTP. 
  • Bilirubin Testing: Bilirubin is the end product that forms when your red blood cells are destroyed. TTP is characterized by hemolytic anemia that increases your bilirubin level. This can help the confirmation of hemolytic anemia.
  • ADAMTS13 Assay: This blood test is especially performed to monitor the level of ADAMTS13 enzyme in the body. This assay confirms the presence of TTP
  • Other Blood Tests: Alongside these, other blood tests such as lactate dehydrogenase test, and Coombs test are used to identify the degree of hemolytic anemia associated with TTP.

Treatment of Thrombotic Thrombocytopenic Purpura (TTP) | When to Consult a Doctor

TTP can last for days or weeks but can be prolonged. Some of the best treatment options against TTP include:

Plasma Therapy

In this treatment, plasma is taken from your blood. Plasma can be given in two ways. Fresh plasma is given to patients who have inherited TTP. Plasma exchange is used for the patients who acquire TTP. Plasma can help to replace the inherited or acquired ADAMTS13 enzyme.

Medication

Medicines are quite helpful to slow down the activity of antibodies that destroy ADAMTS13 enzymes. 

Spleen Removal

This surgical treatment is effective in case of severe TTP. The spleen is the site where platelets destroying proteins are synthesized. So, in the case of severe TTP splenectomy (spleen removal) is an effective way to treat ITP.  

If you experience problems of excessive bleeding and see purple or red spots on your skin, then consult a medical professional as soon as possible.

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