Rituximab for thrombotic thrombocytopenic purpura: benefit of early administration during acute episodes and use of prophylaxis to prevent relapse

• Published in: Journal of thrombosis and haemostasis 2013-03, Vol.11 (3), p.481-490
• Main Authors: Westwood, J‐P., Webster, H., McGuckin, S., McDonald, V., Machin, S. J., Scully, M.
• Format: Article
• Language: English
• Subjects: Acute Disease; ADAM Proteins - blood; ADAMTS13 Protein; Adolescent; Adult; Aged; Antibodies, Monoclonal, Murine-Derived - administration & dosage; Antibodies, Monoclonal, Murine-Derived - adverse effects; Biomarkers - blood; Child; Disease-Free Survival; Drug Administration Schedule; Female; Humans; Immunologic Factors - administration & dosage; Immunologic Factors - adverse effects; Kaplan-Meier Estimate; Male; Medical treatment; Middle Aged; Purpura, Thrombotic Thrombocytopenic - blood; Purpura, Thrombotic Thrombocytopenic - diagnosis; Purpura, Thrombotic Thrombocytopenic - drug therapy; Purpura, Thrombotic Thrombocytopenic - mortality; Recurrence; Registries; Retrospective Studies; Rituximab; Tertiary Care Centers; thrombotic thrombocytopenic purpura; Time Factors; Treatment Outcome; United Kingdom; Young Adult
• ISSN: 1538-7933; 1538-7836; 1538-7836
• DOI: 10.1111/jth.12114

Summary Background Rituximab has been documented in the treatment of acute (≤ 3 days from admission), relapsed/refractory thrombotic thrombocytopenic purpura (TTP) and given as prophylaxis in selected cases to prevent acute relapse. The precise timing of rituximab in acute TTP has not been determined. Objective To perform a retrospective analysis of rituximab use in a large TTP referral center over an 8‐year period. Patients/Methods We assessed response to treatment and outcome for all patients treated with rituximab, including 91 patients presenting with 104 episodes of acute TTP and 15 patients given rituximab as prophylaxis to prevent relapse. In the acute TTP group we assessed the benefit of giving early (≤ 3 days from admission) vs. later (> 3 days) rituximab. Results In acute de novo TTP, previously untreated with rituximab, rituximab was given ≤ 3 days from admission to 54 patients and > 3 days from admission to 32 patients. Earlier administration (≤ 3 days) was associated with faster attainment of remission (12 vs. 20 days, P