Video - Thrombotic thrombocytopenic purpura

Thrombotic thrombocytopenic purpura (TTP) is a rare blood disorder that is characterized by the formation of small blood clots in the small blood vessels throughout the body. These clots can limit or block blood flow to vital organs, leading to tissue damage and organ dysfunction. TTP can also cause low levels of platelets (thrombocytopenia), which are important for blood clotting.

Although the exact cause of TTP is unknown, it's associated with a deficiency of ADAMTS13, an enzyme that breaks down the clotting protein called von Willebrand factor. Without sufficient levels of ADAMTS13 in the blood, large pieces of von Willebrand factor can move around the blood and increase platelet clotting and red blood cell destruction. TTP can be either congenital or acquired. Congenital TTP is caused by a genetic mutation that affects the production or function of ADAMTS13, while acquired TTP is often caused by the development of autoantibodies that target and destroy ADAMTS13.

TTP can cause a wide range of other symptoms such as easy bruising and bleeding, fever, weakness, fatigue, and extreme paleness. There can also be episodes of unusually heavy bleeding or abdominal pain accompanied by nausea and vomiting. Treatment typically involves plasmapheresis, steroids, and other drugs such as rituximab. Plasmapheresis is the process of exchanging plasma through a machine.