| | Therapy | Monitoring | Routine care | Comments |
| First week | - Continue glucocorticoids, rituximab, and caplacizumab (if used)
| - Symptom assessment daily
- CBC daily
- LDH at time of CBC
| - Removal of central venous catheter
| - The highest risk of exacerbation is during the first week.
- Exacerbations are treated with TPE, glucocorticoids, rituximab, and sometimes caplacizumab.
|
| First month (assuming ADAMTS13 activity has increased to >20 to 30%) | - Taper and discontinue glucocorticoids
- Complete rituximab
- Discontinue caplacizumab
| - Symptom assessment (ongoing)
- CBC weekly, then every other week
- ADAMTS13 activity weekly
- LDH and creatinine with CBC until they become normal
| - Provision of wallet card/letter
- Resumption of normal activities
|
| First and second year (assuming clinically stable with ADAMTS13 activity >20 to 30%) | | - ADAMTS13 activity every three months
| - Return to primary care clinician
- Updating of vaccinations
- Seek medical attention (and immediate CBC) for any return of symptoms
| - The risk of relapse is greater during the first two years and greater for those with ADAMTS13 <10% (risk of 20 to 30%), but relapse may not occur imminently (median time to relapse: 5 to 9 years).
- For ADAMTS13 <20%, prophylactic rituximab as a single dose followed by retesting of ADAMTS13 is reasonable.
- For frequent relapses with persistent severe ADAMTS13 activity <20%, splenectomy, maintenance rituximab once every 3 months for 2 to 3 years, or other immunosuppressive agents are reasonable options.
|
| Subsequent years | | - ADAMTS13 activity annually
| - Routine primary care
- Annual hematologist visit
- Monitoring for and treatment of long-term complications of TTP
- Seek medical attention (and immediate CBC) for any return of symptoms
|
