TTP

Thrombotic thrombocytopenic purpura (TTP) is a rare blood disorder characterized by clotting in small blood vessels of the body (thromboses), resulting in a low platelet count.

Signs and symptoms

The classic pentad of symptoms includes thrombocytopenia, microangiopathic hemolytic anemia, neurological abnormalities, fever, and renal disease. However, the full pentad is not always observed.

Causes

TTP can be inherited or acquired. Inherited TTP, also known as Upshaw-Schulman syndrome, is caused by mutations in the ADAMTS13 gene. Acquired TTP is often associated with autoimmune diseases, pregnancy, cancer, HIV infection, and certain medications.

Diagnosis

Diagnosis of TTP is based on clinical findings and laboratory tests. The PLASMIC score can be used to predict the likelihood of TTP in patients with thrombocytopenia and microangiopathic hemolytic anemia.

Treatment

Treatment typically involves plasma exchange and immunosuppressive drugs. In severe cases, rituximab may be used.

Prognosis

With treatment, the majority of people with TTP can lead normal lives. However, relapses can occur.

See also

• Hemolytic-uremic syndrome
• Disseminated intravascular coagulation
• Paroxysmal nocturnal hemoglobinuria

References

TTP Resources
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