From the Research
Patients with thrombotic thrombocytopenic purpura (TTP) face significant long-term mortality risks, with a 30% overall mortality rate and a 2-year overall survival (OS) rate of 68.3%, as reported in a recent study 1. The primary causes of death in TTP survivors include cardiovascular events, recurrent TTP episodes, infections, and malignancies. Approximately 40% of TTP patients experience at least one relapse within 7-10 years of their initial episode, with each recurrence carrying a significant mortality risk. Survivors often develop chronic health complications, including neurocognitive deficits, depression, hypertension, and reduced kidney function. Some key factors associated with the achievement of complete response (CR) in TTP patients include the fall in LDH at D5 of treatment and the daily rhythm of plasma exchange (PE) 1. Long-term outcomes are significantly improved with prompt plasma exchange therapy and immunosuppression during acute episodes, followed by maintenance therapy in selected high-risk patients. Caplacizumab, which prevents platelet aggregation, has shown promise in reducing acute mortality and organ damage 2. Regular monitoring for ADAMTS13 activity levels is essential for early intervention, and patients require lifelong follow-up with hematology specialists, with particular attention to cardiovascular risk management and prompt evaluation of any recurrent symptoms. Some studies have demonstrated that delayed initiation of plasma exchange is associated with increased risk of death and complications, with a gradient of increased mortality risk and thrombotic complications with longer treatment delays 3. However, the most recent and highest quality study 1 provides the most relevant evidence for guiding clinical practice in the management of TTP. Key points to consider in the management of TTP include:
- Prompt initiation of plasma exchange therapy
- Use of immunosuppression during acute episodes
- Monitoring for ADAMTS13 activity levels
- Lifelong follow-up with hematology specialists
- Attention to cardiovascular risk management and prompt evaluation of any recurrent symptoms. The use of plasma exchange and immunosuppression, as well as the monitoring of ADAMTS13 activity levels, are crucial in improving long-term outcomes in TTP patients, with a focus on reducing morbidity, mortality, and improving quality of life 1, 2.