TREATMENT OF THROMBOTIC THROMBOCYTOPENIC PURPURA – OUREXPERIENCE

• Main Author: Matjaž Sever
• Format: Article
• Language: English
• Published: Slovenian Medical Association 2008-04-01
• Series: Zdravniški Vestnik
• Online Access: http://vestnik.szd.si/index.php/ZdravVest/article/view/882

BACKGROUND Thrombotic thrombocytopenic purpura (TTP) is a rare disease. It develops as a consequence of abnormal metabolism of a disintegrin and matalloproteinase with thrombospondincomponents (ADAMTS13). Its clinical picture is heterogenous. Exchange plasmapheresisand various modes of immunosupression are essential for remission induction. Inspite oftreatment only 90 % of patients survive. The purpose of this paper is to present patients’data, clinical picture, treatment and outcome of patients with TTP. METHODS Data of 14 patinets was obtained using the Hipokrat computer program and analysedusing descriptive statistics. RESULTS We treated 14 patients who mainly presented with gastrointestinal and central nervoussystem symptoms and signs. Thirteen patients had idiopathic TTP and one had secondaryTTP. Patients with idiopathic TTP were treated with exchange plasmapheresis and corticosteroids. Primary resistant patients and those with relapses were either splenectomised(5 patients) or received rituximab (3 patients). CONCLUSIONS TTP is a rare disease with a heterogenous clinical presentation. We observed 100 %survival using standard treatment modes and relaps prevention