Steroids
- Higher dose pulsed steroids associated with improved outcomes and minimal side effects
- Usually used in combination with PLEX:
- Intravenous daily methylprednisolone (1 g/day for three consecutive days)
- Oral prednisolone (e.g. 1 mg/kg/day)
Rituximab
- Shown to be effective and safe in refractory immune mediated TTP
- Now recommended for all patients with:
- Cardiac or neurological pathology on admission in conjunction with steroids and PLEX:
- Refractory or relapsing immune-mediated TTP
- Usually given in a dose of 375 mg/m2 has been used weekly for 4 weeks
- PLEX should be withheld for at least 4 h after completing a rituximab infusion