Púrpura trombocitopénica trombótica refractaria

Resumen

Thrombotic thrombocytopenic purpura (TTP) is a rare immune hematologic disease, with an incidence of 1 to 13 per million person-years, in which activity of ADAMTS13 (a vWF-cleaving protease) is severely reduced. We present a case of a 62 years' woman with gastrointestinal and neurologic symptoms along with thrombocytopenia, renal failure and positive anti-ADAMTS13. Treated initialy with plasma apheresis and corticosteroids with good response, she relapsed after two weeks of treatment. Then Rituximab was used with success. In patients treated with plasma apheresis the risk of relapse is high. Newer therapies like Rituximab are effective as we report in this case.