Thrombotic Thrombocytopenic Purpura (Syndroma Moschkowitz), Severe Disease with Potential Fatal Outcome

Thrombotic thrombocytopenic purpura (TTP) is a rare disease with an untreated mortality rate of 90 %. The disease consists of the pentad of microangiopatic hemolytic anemia, thrombocytopenic purpura, neurologic abnormalities, fever and renal failure. TTP, inherited or autoimmune, is mainly caused by the plasma deficiency of the von Wilebrand factor cleaving protease, ADAMTS13, owing to gene mutations or autoantibodies. The prompt delivery of plasma exchange (PEX) has reduced the mortality rate to 10-20 %. Daily PEX should continue for a minimum of 2 days after platelet count has been greater than150 x 10 (9) /l. In refractory cases, the use of anti CD20 monoclonal antibody is recommended. Vincristine also has been shown to be useful in refractory patients.