Dr Rahul Bhargava Haematologist

MBBS, MD, DM (Clinical Haematology)

Principal Director & Chief Hematology at Fortis Hospital

MBBS, MD, DM (Clinical Haematology)

Principal Director & Chief Hematology at Fortis Hospital

Thrombotic thrombocytopenic purpura (TTP)

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Frequently Asked Questions (FAQ) :

TTP can be managed effectively with prompt treatment. While there is no permanent cure, most patients go into remission with appropriate therapy.

 

Untreated TTP can lead to severe complications, including organ failure, stroke, heart attack, and even death.

 

Yes, TTP can relapse in some cases. Continuous monitoring and follow-up care are essential to detect early signs of a relapse.

 

About Thrombotic Thrombocytopenic Purpura (TTP)

Thrombotic Thrombocytopenic Purpura (TTP) is a rare and life-threatening blood disorder. It involves the formation of small blood clots throughout the body, which can damage organs like the heart, brain, and kidneys. These clots form due to a lack of the ADAMTS13 enzyme, leading to low platelet counts, anemia, and other serious health problems. Early detection and treatment of TTP are essential to prevent life-threatening complications.

Causes of Thrombotic Thrombocytopenic Purpura (TTP)

TTP can be triggered by several factors. The main cause is a deficiency in the ADAMTS13 enzyme, responsible for breaking down large protein clumps in the blood. Without this enzyme, small blood clots form and block blood flow to critical organs. Causes include:

  • Inherited: Some individuals are born with a deficiency of the ADAMTS13 enzyme.
  • Acquired: This form occurs when the immune system attacks the enzyme, reducing its levels. This can be triggered by autoimmune diseases, infections, certain medications, or pregnancy.

Types of Thrombotic Thrombocytopenic Purpura (TTP)

TTP is classified into two primary types:

  • Congenital TTP: Also known as Upshaw-Schulman Syndrome, this rare form is inherited and often diagnosed in childhood.
  • Acquired TTP: This type occurs when the immune system attacks and destroys the ADAMTS13 enzyme. It’s more common in adults and can develop suddenly.

Symptoms of Thrombotic Thrombocytopenic Purpura (TTP)

TTP symptoms can appear suddenly and worsen quickly. Common symptoms include:

  • Extreme fatigue and weakness
  • Easy bruising and bleeding (e.g., nosebleeds, bleeding gums)
  • Fever
  • Shortness of breath
  • Confusion or trouble concentrating
  • Headaches
  • Jaundice (yellowing of the skin and eyes)
  • Dark-colored urine
  • Small red or purple spots on the skin (purpura)

Diagnosis of Thrombotic Thrombocytopenic Purpura (TTP)

Diagnosing TTP requires a thorough evaluation. Dr. Bhargava utilizes advanced diagnostic tests, including:

  • Blood tests: To measure platelet levels, hemoglobin, and signs of hemolysis (destruction of red blood cells).
  • ADAMTS13 activity test: To assess enzyme levels and determine if TTP is present.
  • Blood smear: To examine blood cells under a microscope for clumping or abnormal shapes.
  • Kidney and liver function tests: To check for organ damage due to blood clots. Early and accurate diagnosis is key to managing TTP effectively.

Treatment of Thrombotic Thrombocytopenic Purpura (TTP)

Treatment for TTP is aimed at preventing further clot formation and reversing the damage caused by existing clots. Dr. Rahul Bhargava offers personalized treatment options, which may include:

  1. Plasma Exchange Therapy (PLEX): This is the first-line treatment for TTP. Plasma is removed from the blood and replaced with fresh plasma to remove harmful antibodies and replenish the ADAMTS13 enzyme.
  2. Corticosteroids: Medications like prednisone may be prescribed to suppress the immune system and prevent further enzyme destruction.
  3. Rituximab: An immunosuppressive drug that helps reduce antibody production and is often used in patients with acquired TTP.
  4. Caplacizumab: A newer medication that helps prevent clot formation and is used in conjunction with plasma exchange.
  5. Supportive care: This may include blood transfusions, kidney support, and other therapies depending on the patient’s needs.

Cost of Treatment and Stay in India

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