Thrombotic Thrombocytopenic Purpura (TTP)

TTP or Thrombotic Thrombocytopenic Purpura is a rare disease of the group known as thrombotic  microangiopathies. These are diseases  in which the platelets or thrombocytes (involved in coagulation) clog together in an abnormal manner leading to the formation of blood clots.  

This phenomenon causes three problems:

• A reduction in the number of available platelets = Thrombocytopenia.
• The red blood cells collide with these clots and break up leading to a reduction in number = Anaemia.
• The clots can block the blood vessels and reduce the oxygenation of tissues with possible serious consequences for the heart, brain, kidneys, etc.  

A number of causes can be responsible for the occurrence of these thrombotic microangiopathies. The TTP is caused by the deficiency of a protein known as   "Adamts 13". This deficiency is most frequently due to the presence of an antibody (= immune TTP) or otherwise it may be a genetic anomaly (congenital TTP).  

TTP must be treated as a vital emergency. Without treatment the mortality rate is 90%. A rapid diagnosis is essential followed by optimal treatment at an expert centre.  

Initial treatment of immune TTP consists of two action plans:

1.Acute treatment

Increasing the level of Adamts 13 proteins by means of plasma exchanges: The plasma (the liquid component of the blood that contains cells) is replaced with healthy plasma that serves to increase Adamts 13 levels and remove antibodies.    

Since 2019 an innovative medicine specific to TTP has made it possible to prevent the platelets from attaching themselves to each other, thereby resolving the three problems indicated above. This has permitted a clear improvement in the treatment of acute stage patients. Our centre always has this medicine on hand for rapid administration.    

2.Fundamental treatment

Stopping the production of antibodies that act against Adamts 13. First line treatment consists of the administration of corticoids and a monoclonal antibody that targets the cells that produce the antibodies  

Treatment of congenital TTP is based on the administration of plasma. The administration of  synthetic Adamts 13 is not yet reimbursed but is accessible.  

All persons suffering from TTP require lifelong monitoring by a haematologist specialising in this type of pathology.   

TTP can present various and variable symptoms as it can affect a number of organs. The primary clinical manifestation is abnormal bleeding, major hematomas or petechiae (multiple small red/violet marks on the skin).  

More severe symptoms can be the signs of a heart attack or stroke.  

A blood test showing anaemia and a thrombocytopenia can quickly suggest the diagnosis .

Useful links:   

Thrombotic Microangiopathies - MaRIH – Rare Immuno-Haematological Diseases Health Network 

National Reference Centre Microangiopathies 

TTP Community - Home (Dutch language site) 

Our hematology team has gained extensive experience in managing this condition and regularly receives requests for the care of patients from external centers.

We regularly collaborate with expert centers from various European countries (France, United Kingdom, Italy).

We participate in multidisciplinary consultation meetings with French expert centers.