hematology and oncology
Thrombotic Thrombocytopenic Purpura (TTP)
Key Points
Platelets and red blood cells are destroyed nonimmunologically by microvascular thrombi, leading to thrombocytopenia, anemia, and organ ischemia. The cause is deficient activity of theADAMTS13protease, which is usually due to an acquired autoantibody but rarely by an inherited gene mutation. Untreated thrombotic thrombocytopenic purpura is usually fatal. Timely treatment with plasma exchange along with corticosteroids andrituximabresults in > 85% survival rates. 