Bortezomib therapy in patients with relapsed/refractory acquired thrombotic thrombocytopenic purpura

Therapeutic plasma exchange (TPE) has dramatically improved the prognosis of acquired thrombotic thrombocytopenic purpura (TTP), and TPE and corticosteroids are the mainstays of treatment of acquired TTP. However, a subset of patients may remain refractory to this treatment modality, or have an initial response but relapse after the discontinuation of TPE during the follow-up. While managing patients with relapsed/refractory acquired TTP, there are several therapeutic maneuvers, which can be considered. Recently, there came some papers regarding the use of bortezomib in relapsed or refractory acquired TTP, and in this review, this indication of bortezomib was evaluated along with the current data available. In seven published papers, 12 patients with relapsed/refractory acquired TTP received bortezomib, of which 11 survived the acute episodes and maintained remission. Bortezomib may serve as an adjunct treatment, but prospective trials are needed to determine the dosing, administration route, and the treatment schedule of this treatment option in patients with relapsed/refractory TTP.