Clinical Presentation and Follow-up of Eighteen Patients with Thrombotic Thrombocytopenic Purpura
Background and Objectives: Major advances achieved during the past 30years in under-standing the pathophysiology of Thrombotic Thrombocytopenic Purpura (TTP). More recently treatment outcomes were revolutionized by the introduction of plasma exchange. In this study a descriptive analysis of clinical presentation and treatment outcomes with a modified Plasma Exchangee protocol (PE) is presented.
Methods: During the period from November 2004 to July 2006, eighteen patients with acute episode of TTP were enrolled. After clinical and Laboratory evaluation, patients were assigned to receive modified “low volume plasma exchange “, and their response was monitored for a mean of 64 weeks.
Results: The classical Pentad of TTP was only documented in 27% cases. Of patients enrolled, one died before starting PE, while of the remaining 17 put on PE Complete remission (CR) was achieved in 70% with a mean follow up of 64 weeks. Four patients died during the first few days of starting PE. Of patients who achieved CR none had a relapse.
Conclusions: The classical “pentad” of TTP is present in small proportion of patients at presentation. Our results with the modified “low volume exchange ” may suggest that even small volume of plasma exchange can be life saving .
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