From the Research
Long-term outcomes for patients with thrombotic thrombocytopenic purpura (TTP) are significantly improved with prompt treatment, with a 2-year overall survival rate of 68.3% and a relapse-free survival rate of 84.4%. The most recent and highest quality study 1 suggests that plasma exchange (PE) is an effective treatment for TTP, with a complete response rate of 69% and an overall mortality rate of 30%. Factors associated with the achievement of complete response include the fall in LDH at D5 of treatment and the daily rhythm of PE.
Key Findings
- The study 1 included 26 patients with TTP, with a median age of not specified, and found that PE was initiated within a median of 1 day.
- The rhythm of exchanges was daily in 22 patients, and 20 PE-related complications were noted, with hypocalcemia being the most frequent (30%).
- Complete response was achieved in 15 patients after PE alone, and 9 patients were refractory, with 6 receiving 2nd-line treatment, and 5 achieving complete response.
- Relapse was noted in 6 patients (40%), and they were treated with PE, with only 1 patient receiving rituximab, and 4 patients having a response.
Long-term Management
- Long-term management of TTP typically involves regular monitoring of ADAMTS13 activity levels every 3-6 months, with rituximab (375 mg/m² weekly for 4 weeks) sometimes used prophylactically when levels fall below 10-15% 1.
- Some patients with recurrent relapses may benefit from maintenance therapy with rituximab every 6-12 months or immunosuppressants like mycophenolate mofetil (500-1000 mg twice daily).
- The importance of comprehensive long-term follow-up care cannot be overstated, as survivors of TTP face increased risks of cognitive impairment, depression, hypertension, and reduced quality of life.
Complications and Risks
- Neurocognitive deficits are particularly common, affecting up to 60% of patients, and manifesting as memory problems, concentration difficulties, and processing speed reductions.
- Cardiovascular complications include a higher incidence of stroke, myocardial infarction, and hypertension compared to the general population.
- Chronic kidney disease develops in about 20-30% of patients, while depression and anxiety affect approximately 40%.