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  • Zdravljenje kronične idiopatične trombotične trombocitopenične purpure s splenektomijo = Management of chronic idiopathic thrombotic thrombocytopenic purpura with splenectomy
    Modic, Mojca, 1954- ; Zver, Samo, 1966-
    Background. Treatment of thrombotic thrombocytopenic purpura (TTP) with plasmaexchange (PE) has reduced mortality rates from 90% in untreated patientsto less than 20%. Despite primary effective PE, ... relapses occur in up to 40%. Despite primary effective PE, relapses occur in up to 40% of patients.Beside PE, second line treatment options are corticosteroids, vincristine, defibrotide, prostacycline. We describe two patients with relapsing TTP treated successfully with splenectomy. Patients and methods. Case 1. A 20-year female was diagnosed in October 2000 with idiopathic chronicrelapsing TTP. First episode of PE plus methylprednisolone treatment led to complet remission. Subsequent relapses occured 3, 6 and 8 months after first TTP episode. Every relaps was effectivelly treated with PE and methylprednisolone. Because of more and more frequent TTP relapses laparoscopic splenectomy was performed. Currently, 7 months after splenectomy she is in complet TTP remission. Case Ž. A 18-year female, was diagnosed in January 1997 with idiopathic TTP. After PE and methylprednisolone complete remission was established. Relaps occured after nearly three years. Later on, TTP got chronic course with neurological clinical symptoms and plateled drops as soon as PE treatment was withdrawn. Besides PE and methylprednisolone she received also defibrotide. Because of chronic, PE dependant TTP, laparoscopic splenectomy was performed. Today, 15 months after splenectomy she is in complete TTP remission. Conclusions. In idiopathic relapsing and chronic TTP, PE treatment only is usually not sufficient for durable remission. Second lineTTP treatment drugs have limited efficacy. Based on our experience in patients with relapsing and/or chronic TTP, laparoscopic splenectomy should beconsidered as a first treatment option, especialy in junger patients.
    Vrsta gradiva - članek, sestavni del
    Leto - 2002
    Jezik - slovenski
    COBISS.SI-ID - 15207129