Plasma exchange (mainstay of treatment; ideally started within 4-8 hours of initial clinical diagnosis) and methylprednisolone (1 g/day IV for 3 days for adults) or high-dose oral prednisolone (for example, 1 mg/kg/day) and (in some cases) rituximab or caplacizumab; for secondary TTP, same treatment as with primary TTP in addition to treatment of the underlying disorder.
Guideline recommendations:
ISTH guidelines for treatment of thrombotic thrombocytopenic purpura:
British Committee for Standards in Haematology (BCSH) guidelines on diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies:
