- Should be initiated as soon as possible (preferably within 4-8 hours of diagnosis):
- Early initiation associated with better outcomes
- May require emergency transfer to specialist centre
- Should not wait for result of ADAMTSq3 activity assay
- Ideally daily spun apheresis should be performed:
- Initially, 1.5x plasma volume should be exchanged for 3 days
- Then 1x plasma volume can be exchange
- Once platelet count >150 x 109/L for 2 days consecutively can be discontinued
- Duration of PLEX required to achieve remission is often highly variable
- Detergent treated FFP should be used to reduce the risk of transfusion-transmitted infection
