What is the recommended treatment for newly diagnosed thrombotic thrombocytopenic purpura (TTP)?

By William Aird

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:

The panel was unable to make a more detailed recommendation on a preferred dosage and type of corticosteroids (eg, prednisone, or methylprednisolone).
The data informing this recommendation are from nonrandomized trials.

British Committee for Standards in Haematology (BCSH) guidelines on diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies: