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Thrombotic Thrombocytopenic Purpura (TTP)

What is thrombotic thrombocytopenic purpura?

Thrombotic thrombocytopenic purpura (TTP) is a rare blood disorder that is characterized by blood clots that form in small blood vessels. These clots can be dangerous if they block blood flow to the brain, heart, or other major organ. 

There are two types of this condition: acquired and familial. The first sees episodes of symptoms, which can appear in childhood or adulthood. An affected individual may experience just one or multiple episodes. The second form sees more constant symptoms and tends to appear in infancy or childhood, although onset can occur in adulthood as well. 

While the incidence of this disorder is unknown, medical professionals do know that it is more likely to impact females than males. 

What are the symptoms of thrombotic thrombocytopenic purpura?

Blood clots are the major symptom of this condition. If they block blood flow to any major organ, they can result in serious complications, including neurological problems, abnormal kidney function, heart problems, fever, and abdominal pain. 

Thrombocytopenia, or a low platelet count, is another symptom. This can lead to purpura, or small spots of bleeding under the skin that appear as purplish areas. Red blood cells are destroyed in this condition as well, and if they are destroyed faster than they are created, then affected individuals will experience hemolytic anemia. This results in fatigue, jaundice, a rapid heart rate, paleness, and shortness of breath. 

What causes thrombotic thrombocytopenic purpura?

The familial form is caused by an autosomal recessive mutation in the ADAMTS13 gene, which leads to a reduction in activity of an enzyme required for proper blood clotting. The same enzyme is impacted in those with the acquired form, except autoantibodies attack it. 

How is thrombotic thrombocytopenic purpura diagnosed?

Doctors will first ask for a patient’s medical history, followed by a physical exam. Tests are also necessary, including a complete blood count, platelet count, kidney function and urine test, blood smear, Coombs test, Bilirubin test, lactate dehydrogenase test, and an ADAMTS13 assay. 

What are the treatments for thrombotic thrombocytopenic purpura?

It is important to seek treatment for TTP when an episode occurs, as severe complications can occur. Plasma therapy is the main treatment for this condition. 

Where can I find out more about thrombotic thrombocytopenic purpura?

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