Treatment of Adults with Clinically Suspected Severe Thrombotic Thrombocytopenic Purpura – Experiences of a Single Centre

Thrombotic thrombocytopenic purpura (TTP), even though uncommon, has an increasing prevalence and is fatal if untreated. Plasma exchange (PE), if started within 48 h of presentation, has improved the clinical outcome and reduced the mortality rate to 10-35%. Major complications and allergic reactions related to PE are reported to occur in about 50% of patients and catheter-related complications in up to 30% of patients. To further reduce mortality and relapses in patients with TTP, therapeutic strategies should aim at minimizing complications related to treatment. Patients and Methods: A total of 31 patients with clinically suspected severe TTP were treated with the following protocol: i) pre-medication with steroids and antihistamines just before each PE procedure; ii) continuous calcium gluconate substitution; iii) insertion of a jugular central venous catheter using the Seldinger technique under ultrasonic guidance; iv) continuous low-dose anti-coagulation treatment via the central venous catheter. Results: No treatmentrelated mortality occurred. In 434 PE procedures, none of our patients developed allergic reactions or major complications. The incidence of minor complications was less than 10%. Our insertion technique has a low complication rate of 14%. The centrifugal plasma separation technique prevents thrombocyte losses. Over an median follow-up of 3.5 years, the relapse rate amounted to 3%. Conclusion: Our protocol presented here allows a safe treatment of severe TTP with a low incidence of complications and a low relapse rate.