Thrombotic thrombocytopenic purpura (TTP) is a rare severe disease that must be diagnosed and treated promptly for a successful outcome. We report a case of severe acute TTP treated with plasma exchange and rituximab. The patient presented at diagnosis with severe microangiopathic haemolytic anemia, jaundice, fever, seizures followed by coma and hypovolemic shock. Her LDH was 2,860 IU/L; platelet count 37 x 10(9)/L; hemoglobin 5.1 g/dL; blood smears contained schistocytes. Treatment for TTP was started with pulses of methylprednisolene and daily single-volume plasma exchange using fresh frozen plasma. After five plasma exchanges, the neurological status worsened, LDH increased sharply as did ALT and AST and the platelet count dropped to 72 x 10(9)/L. Rituximab (weekly doses of 375 mg/m² for 4 weeks) and plasma exchange with cryosupernatant substitution fluid were initiated. Within two days there was neurological improvement, stable platelet levels and decreased LDH. Complete remission, achieved after 23 plasma exchanges and four doses of rituximab, has been maintained for 34 months. Plasma exchange with cryosupernatant substitution fluid and rituximab played an important role in the successful treatment of this patient with severe acute TTP. A systematic clinical trial should be considered in order to confirm these findings.
Thrombotic thrombocytopenic purpura; plasma exchange; rituximab; hemolytic anemia; therapy
