Abstract

Objective and importanceThrombotic thrombocytopenic purpura (TTP) is a life-threatening disorder that needs prompt diagnosis and treatment. Front-line therapy consists of plasma exchange (PEx) and steroids, but, in some instances, this is not enough to achieve a complete and sustained response.Clinical presentationWe report four cases of TTP treated with low-dose rituximab, PEx, and a short course of steroids with an excellent outcome. Three of the patients had primary TTP and another presented an underlying human immunodeficiency virus infection.InterventionRituximab, 100 mg intravenously, was initiated on days 2–8 from the start of PEx as first-line therapy in three cases and as salvage therapy for relapsing disease in one. The number of PEx needed ranged from 5 to 12 sessions. All patients achieved complete remission and are currently asymptomatic, with complete response duration of 8–22 months.ConclusionTreatment of TTP with low-dose rituximab, along with PEx and steroids, seems to be as effective as the standard dose of monoclonal antibody.

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