• 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