What are the signs of TTP
Thrombotic thrombocytopenic purpura (TTP), Moschcowitz disease
It is a multisystem disease with thrombocytopenia, hemolytic anemia, neurological abnormalities, often with renal insufficiency and fever.
The maximum age of the disease is around the age of 40, women are affected about twice as often as men. The incidence is around 3-10 in a million per year.
Etiology and pathogenesis
In patients with TTP, unusually large multimers of the von Willebrand factor are found, which induce platelet aggregation via the glycoprotein Iba component of the platelet glycoprotein complex Ib / IX / V. The cause of the formation of the large multimers is due to a reduced activity of the metalloprotease enzyme, which is known as ADAMTS13(e-disintegrin and metalloprotease with thrombospontin-I-like domains) referred to as. The cause of the acquired, more common form is the formation of inhibiting autoantibodies. A gene mutation in the ADAMTS13 gene on chromoson 9q34 is responsible for the familial form. The activity of this enzyme is less than 10% in the acute episode of the disease. In about 15% of all affected patients, the disease is stimulated by various causes. Since most multimers originate from the endothelium, damage to the endothelial cells leads to the multiplication of the multimers in the plasma.
This is characteristic of the disease Triad out:
In addition, more than half of the patients show signs of renal insufficiency and occasionally petechial skin changes. Some of the patients (approx. 25%) complain of a fever.
Untreated thrombotic thrombocytopenic purpura used to lead to death in almost all cases. A TTP recognized and treated in good time has a good chance of permanent complete remission, but even today up to 10% of patients die. In up to 60% of cases, relapses are possible, which in turn can be treated.
Current reports from the 58th
ASH Annual Meeting 2016,
San Diego, California, USA [more]
2016 ASCO Annual Meeting - current reports. This service is funded by:
Editor: Prof. Dr. H. Link
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