For acquired TTP treatment is recommended as follows:
1. For patients with probable TTP it is recommended that treatment withplasma exchange (PE) be initiated as soon as possible. PE of 1.5 PV should be carried out until remission however, if responseslows or if there is no change, increase daily PE until response. NOTE: the choice of the plasma replacement fluid should be at thediscretion of the treating physician and should be chosen from CSP, SDP, Frozen Plasma or other suitable fluid, without restriction and asrequired by the patient.
2. Steroids may be added to the protocol at disease onset or if no/orslowing response, generally at a dose of 1 mg/kg prednisone orally.If severe end-organ dysfunction is present (e.g. stroke, seizures,myocardial infarction) consider pulsed IV methylprednisolone daily forthree days then reduce to 1 mg/kg prednisone or equivalent.
3. Rituximab should be considered in refractory TTP (no response after5 PE or return of disease in 0 to 30 days post remission).
4. If disease continues to progress, consider rescue therapy with T cellsuppression (cyclophosphamide, cyclosporine), plasma cell suppression(bortezomib) or, if disease continues, a
combination (+/- splenectomy).
5. Rituximab should also be used in patients who achieved remission butwho have subsequently relapsed (i.e. return of disease at least 30 daysafter the last PE).
Canadian Apheresis Group (CAG) TMA Algorithm