Zdravljenje trombotične trombocitopenične purpure - naše izkušnje
Treatment of thrombotic thrombocytopenic purpura - our experience

slovenian

2008

volume 77, issue 1

Purpura, Thrombotic Thrombocytopenic   Splenectomy   Therapy

Slovensko zdravniško društvo
(Obvezni izvod spletne publikacije)

Background Thrombotic thrombocytopenic purpura (TTP) is a rare disease. It develops as a consequence of abnormal metabolism of a disintegrin and matalloproteinase with thrombospondin components (ADAMTS13). Its clinical picture is heterogenous. Exchange plasmapheresis and various modes of immunosupression are essential for remission induction. Inspite of treatment only 90% of patients survive. The purpose of this paper is to present patientsć data, clinical picture, treatment and outcome of patients with TTP. Methods Data of 14 patinets was obtained using the Hipokrat computer program and analysed using descriptive statistics. Results We treated 14 patients who mainly presented with gastrointestinal and central nervous system symptoms andsigns. Thirteen patients had idiopathic TTP and one had secondary TTP. 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.

16.09.2009