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Clinically, this condition is associated with thrombotic microangiopathy, a disorder characterized by the pathological formation of platelet aggregates (thrombi) in the small blood vessels throughout the body.
In thrombotic thrombocytopenic purpura, this results in:
- Reduction of platelets due to their excessive consumption (which is why it is called "thrombocytopenic": these cells are used for blood clotting);
- Mechanical damage to erythrocytes (haemolytic anemia);
- Neurological symptoms.
The presentation is often acute and very serious: if the TTP is not recognized and treated, the episode can escalate to a coma and lead to death in 90% of cases.
The treatment of choice for thrombotic thrombocytopenic purpura is plasma exchange (ie the replacement of the patient's plasma with that from donors, but, sometimes, it is necessary to consider immunosuppressive therapy or other interventions.
haemolytic and neurological disorders, impaired renal function and fever.
Thrombocytopenia is caused by the reduced enzymatic activity of ADAMTS 13. This deficiency can be secondary to mutations in the gene coding for the protein (hereditary form) or it can be acquired during life, usually due to the body's own production of antibodies. that target this protein (auto-immune form).
In any case, the consequence of this dysfunction is the formation of thrombus in the small blood vessels of the whole organism (which is why it is called thrombotic).
Throughout life, thrombotic thrombocytopenic purpura episodes can be single or repeated.
