What causes thrombotic thrombocytopenia purpura?
Causes. A lack of activity in the ADAMTS13 enzyme (a type of protein in the blood) causes thrombotic thrombocytopenic purpura (TTP). The ADAMTS13 gene controls the enzyme, which is involved in blood clotting. Not having enough enzyme activity causes overactive blood clotting.
What is thrombotic thrombocytopenia purpura?
Thrombotic thrombocytopenic purpura (TTP) is a blood disorder in which platelet clumps form in small blood vessels. This leads to a low platelet count (thrombocytopenia).
What is purpura associated with?
Ecchymoses or bruises are larger extravasations of blood. Palpable purpura is purpura than can be felt, due to inflammation of the blood vessels (vasculitis) Pigmented purpura is a sign of petechial haemorrhages associated with capillaritis.
Does Purpura cause anemia?
These pinpricks are usually called petechiae or purpura, depending on their size. It is most commonly attributed to a low platelet count, which can be seen in aplastic anemia or with certain infections. Anemia usually refers to a shortage of red blood cells in the body.
How is thrombocytopenic purpura diagnosed?
How is idiopathic thrombocytopenic purpura diagnosed?
- Complete blood count (CBC). A measurement of size, number, and maturity of different blood cells in a specific volume of blood (to measure platelets.
- Additional blood and urine tests.
- Careful review of your medications.
How anti ADAMTS13 cause thrombotic thrombocytopenic purpura?
Congenital TTP is caused by a defect in the ADAMTS13 gene resulting in decreased or absent enzyme activity; acquired TTP results from autoantibodies that either inhibit the activity or increase the clearance of ADAMTS13.
Is thrombotic thrombocytopenic purpura autoimmune?
The most common form of thrombotic thrombocytopenic purpura (TTP), this autoimmune disorder affects up to 10,000 people in the US and Europe annually. Unless acquired autoimmune TTP is recognized and treated promptly, mortality approaches 90%.