TREATMENT OF THROMBOTIC THROMBOCYTOPENIC PURPURA – OUREXPERIENCE
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 immunosupress...
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| Format: | Article |
| Language: | English |
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Slovenian Medical Association
2008-04-01
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| Series: | Zdravniški Vestnik |
| Online Access: | http://vestnik.szd.si/index.php/ZdravVest/article/view/882 |
| Summary: | 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 |
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| ISSN: | 1318-0347 1581-0224 |