Thrombotic thrombocytopenic purpura: Get informed

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Thrombotic thrombocytopenic purpura (TTP)

What is Thrombotic Thrombocytopenic Purpura (TTP)? 

Definition

Thrombotic thrombocytopenia purpura (TTP) is a serious and rare blood disorder in which platelets cause clots. The tiny clots can block blood vessels, which stops the blood from being able to reach the organs. This can compromise the functioning of vital organs such as heart, brain, and kidneys… 

  • “Thrombotic” refers to the blood clots that form.
  • “Thrombocytopenic” means the blood has a lower-than-normal platelet count.
  • “Purpura” refers to purple bruises caused by bleeding under your skin.

TTP may be either congenital (cTTP) or acquired (iTTP for immune TTP). As TTP transmits as an autosomal recessive trait. The parents don’t usually have symptoms of TTP. 

Symptoms of Thrombotic Thrombocytopenic Purpura (TTP)

The symptoms of TTP are caused by blood clots, a low platelet count, and damaged red blood cells. The signs and symptoms include: 

  • Purpura: Purplish bruises on the skin or mucous membranes (such as in the mouth). These bruises are caused by bleeding under the skin,
  • Petechiae:  Pinpoint-sized red or purple dots on the skin. Bleeding under the skin is what causes petechiae,
  • Paleness,
  • Fatigue (weakness),
  • Anemia,
  • A fast heart rate or shortness of breath,
  • Neurologic disturbances like Headache, speech changes, confusion, coma, stroke, or seizure,
  • Fever and A low amount of urine, or protein or blood in the urine may also happen.

Causes and risk factors of Thrombotic Thrombocytopenic Purpura (TTP)

TTP is associated with a deficiency of an enzyme called ADAMTS13, a protease involved in blood clotting. The deficiency of this enzyme allows large complexes of the clotting protein known as von Willebrand factor to circulate in the blood, resulting in platelet clotting and the destruction of red blood cells. 

The disease affects twice as many women as men and can develop during pregnancy causing serious complications. Acquired TTP mostly occurs in adults, but it can affect children while inherited TTP mainly affects newborns and children. Other medical conditions such as Cancer, HIV, lupus, infections and some medicines (chemotherapy, ticlopidine, clopidogrel, cyclosporine A, and hormone therapy) may raise the risk of TTP. 

Diagnosing Thrombotic Thrombocytopenic Purpura (TTP)

The diagnosis of TTP starts with a Complete blood count (CBC), peripheral blood smear, reticulocyte count, serum LDH and renal function tests. The diagnosis is confirmed by the finding of severely deficient (<10%) ADAMTS13 activity for both cTTP and iTTP, and the presence of an anti-ADAMTS13 antibody in patients with iTTP

Early recognition is important in order to initiate treatment as quickly as possible.

Treating Thrombotic Thrombocytopenic Purpura (TTP) 

Regardless of whether it is inherited (congenital) or acquired (immune), TTP can cause life-threatening complications if it is not treated right away. Plasma treatments and medicines are the most common ways to treat TTP. If these treatments do not work, surgery may be needed. Treatments are done mostly in a hospital. 

For congenital (inherited) TTP

Plasma infusion:  Donor plasma is administered intravenously (IV) to replace the missing or defective ADAMTS13 enzyme. 

For immune (acquired) TTP

Therapeutic plasma exchange (plasmapheresis): the patient plasma is replaced with donor plasma. It will not only remove the antibodies that attack the ADAMTS13 protease, but it will also replace the ADAMTS13 enzyme. 

Corticosteroids: Steroids are believed to suppress the production of anti-ADAMTS13 autoantibodies and should be started upfront together with PEX for the acute management of acquired TTP 

Rituximab: Rituximab, a monoclonal antibody that targets the CD20 antigen present on B lymphatic cells, is increasingly being used as a first-line treatment for acute episodes of TTP as well as to prevent relapses and sustain a long-term response. 

Caplacizumab: Caplacizumab, a humanized monoclonal antibody fragment, prevents the platelets adhesion. It is today approved for the treatment of TTP. It should be prescribed for patients with a clinical presentation of TTP and who have a confirmed ADAMTS13 activity level. 

Surgery to remove your spleen: The spleen is an organ in the upper left abdomen. The spleen makes the antibodies that block ADAMTS13 enzyme activity. Removing the spleen stops the body from making these antibodies. This surgery is used to treat TTP if other treatments do not work. 

Living with Thrombotic Thrombocytopenic Purpura (TTP) 

Some people fully recover from thrombotic thrombocytopenic purpura (TTP). However, relapses can occur with many people who have acquired and inherited TTP.  Therefore, some aspects must be considered in order to avoid worsening your TTP.

Over-the-counter medications such as vitamins, herbal supplements, or those containing NSAIDs like aspirin and ibuprofen can prevent platelets from working properly. They should be avoided without consulting a physician.

Food and drinks that contain quinine are always to avoid. Please, read all food and drink labels to check for quinine and ask your healthcare provider for a full list of drinks and foods that contain quinine.

Play it safe as it’s better to avoid activities that could cause injury. Contact sports, such as boxing, martial arts, and football, present a high risk of injury.

Finally, patients who have had a splenectomy should be particularly vigilant for signs of infection, such as fever, and must be taken care of promptly if they develop the symptoms. 

Published Jul 29, 2022

avatar Hela Ammar

Author: Hela Ammar, Pharmacist, data scientist

Hela is a PharmD and holds a master's degree in Pharmaceutical and Biotechnology Management from ESCP Business School. Through her various experiences, Hela has acquired a transversal vision of the health field and... >> Learn more

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