title: Preemptive rituximab infusions after remission efficiently prevent relapses in acquired thrombotic thrombocytopenic purpura.
abstract: In acquired thrombotic thrombocytopenic purpura (TTP), the persistence of severe ADAMTS13 deficiency (<10%) during remission is associated with more relapse. Preemptive (ie, after remission) administration of rituximab in these patients to prevent relapses remains controversial. We performed a cross-sectional analysis of 12-year follow-up data to compare the relapse incidence with or without preemptive rituximab infusion. Among 48 patients who experienced at least one episode of acquired TTP followed by severe ADAMTS13 deficiency during remission, 30 received preemptive rituximab (group 1); the other 18 did not (group 2). After a median of 17 months (interquartile range [IQR], 11-29) following rituximab, the relapse incidence decreased from 0.57 episodes/year (IQR, 0.46-0.7) to 0 episodes/year (IQR, 0-0.81) (P < .01) in group 1. ADAMTS13 activity 3 months after the first rituximab infusion increased to 46% (IQR, 30%-68%). Nine patients required additional courses of rituximab. In 5 patients, ADAMTS13 activity failed to increase durably. Four patients experienced manageable adverse effects. In group 2, the relapse incidence was higher (0.5 relapses/year; IQR, 0.12-0.5; P < .01). Relapse-free survival was longer in group 1 (P = .049). A persistent severe ADAMTS13 deficiency during TTP remission should prompt consideration of preemptive rituximab to prevent relapses.
authors.affiliation: Centre Hospitalier Universitaire La Pitié-Salpétrière, Service de Médecine Interne, Assistance Publique-Hôpitaux de Paris, Paris, France; Université Pierre et Marie Curie Sorbonne Universités, Université Paris 06, Paris, France;
authors.forename: Miguel
authors.initials: M
authors.lastname: Hie
authors.affiliation: Université Pierre et Marie Curie Sorbonne Universités, Université Paris 06, Paris, France; Centre Hospitalier Régional Saint-Antoine, Service d'Hématologie, AP-HP, Paris, France;
authors.forename: Julie
authors.initials: J
authors.lastname: Gay
authors.affiliation: Service d'Immunologie Clinique, Hôpital Saint-Louis, Assistance Publique - Hôpitaux de Paris, Université Paris Diderot, Sorbonne Paris Cité, Paris, France; Centre de Référence des Microangiopathies Thrombotiques, Hôpital Saint-Antoine, Assistance Publique - Hôpitaux de Paris, Paris, France;
authors.forename: Lionel
authors.initials: L
authors.lastname: Galicier
authors.affiliation: Centre de Référence des Microangiopathies Thrombotiques, Hôpital Saint-Antoine, Assistance Publique - Hôpitaux de Paris, Paris, France; Hôpital Albert Calmette, Service de Néphrologie, Lille, France;
authors.forename: François
authors.initials: F
authors.lastname: Provôt
authors.affiliation: Centre de Référence des Microangiopathies Thrombotiques, Hôpital Saint-Antoine, Assistance Publique - Hôpitaux de Paris, Paris, France; Service de Néphrologie-Médecine Interne, Hôpital Sud, Amiens, France;
authors.forename: Claire
authors.initials: C
authors.lastname: Presne
authors.affiliation: Centre de Référence des Microangiopathies Thrombotiques, Hôpital Saint-Antoine, Assistance Publique - Hôpitaux de Paris, Paris, France; Centre Hospitalier Régional de Marseille Conception, Service d'Hémaphérèse, Marseille, France;
authors.forename: Pascale
authors.initials: P
authors.lastname: Poullin
authors.affiliation: Centre de Référence des Microangiopathies Thrombotiques, Hôpital Saint-Antoine, Assistance Publique - Hôpitaux de Paris, Paris, France; Centre Hospitalier Universitaire Charles Nicolle, Service de Réanimation, Rouen, France;
authors.forename: Guy
authors.initials: G
authors.lastname: Bonmarchand
authors.affiliation: Centre de Référence des Microangiopathies Thrombotiques, Hôpital Saint-Antoine, Assistance Publique - Hôpitaux de Paris, Paris, France; Centre Hospitalier Maison Blanche, Service de Néphrologie, Reims, France;
authors.forename: Alain
authors.initials: A
authors.lastname: Wynckel
authors.affiliation: Centre de Référence des Microangiopathies Thrombotiques, Hôpital Saint-Antoine, Assistance Publique - Hôpitaux de Paris, Paris, France; Centre Hospitalier Universitaire Charles Nicolle, Service de Médecine Interne, Rouen, France;
authors.forename: Ygal
authors.initials: Y
authors.lastname: Benhamou
authors.affiliation: Centre de Référence des Microangiopathies Thrombotiques, Hôpital Saint-Antoine, Assistance Publique - Hôpitaux de Paris, Paris, France; Centre Hospitalier de Valenciennes, Service de Néphrologie, Valenciennes, France;
authors.forename: Philippe
authors.initials: P
authors.lastname: Vanhille
authors.affiliation: Centre de Référence des Microangiopathies Thrombotiques, Hôpital Saint-Antoine, Assistance Publique - Hôpitaux de Paris, Paris, France; Centre Hospitalier Universitaire Necker-Enfants Malades, Service de Néphrologie, Assistance Publique - Hôpitaux de Paris, Paris, France;
authors.forename: Aude
authors.initials: A
authors.lastname: Servais
authors.affiliation: Centre de Référence des Microangiopathies Thrombotiques, Hôpital Saint-Antoine, Assistance Publique - Hôpitaux de Paris, Paris, France; Centre Hospitalier Universitaire Dupuytren, Service d'Hématologie Clinique et de Thérapie Cellulaire, Limoges, France;
authors.forename: Dominique
authors.initials: D
authors.lastname: Bordessoule
authors.affiliation: Centre de Référence des Microangiopathies Thrombotiques, Hôpital Saint-Antoine, Assistance Publique - Hôpitaux de Paris, Paris, France; Centre Hospitalier Le Mans, Service de Néphrologie, Le Mans, France;
authors.forename: Jean-Philippe
authors.initials: JP
authors.lastname: Coindre
authors.affiliation: Centre de Référence des Microangiopathies Thrombotiques, Hôpital Saint-Antoine, Assistance Publique - Hôpitaux de Paris, Paris, France; Hôpital Hôtel-Dieu, Service Médecine Interne A, Nantes, France;
authors.forename: Mohamed
authors.initials: M
authors.lastname: Hamidou
authors.affiliation: Université Pierre et Marie Curie Sorbonne Universités, Université Paris 06, Paris, France; Centre de Référence des Microangiopathies Thrombotiques, Hôpital Saint-Antoine, Assistance Publique - Hôpitaux de Paris, Paris, France; Centre Hospitalier Universitaire la Pitié-Salpétrière, Service d'Hématologie, Assistance Publique - Hôpitaux de Paris, Paris, France;
authors.forename: Jean-Paul
authors.initials: JP
authors.lastname: Vernant
authors.affiliation: Centre de Référence des Microangiopathies Thrombotiques, Hôpital Saint-Antoine, Assistance Publique - Hôpitaux de Paris, Paris, France; Service d'Hématologie Biologique, Hôpital Antoine Béclère, Assistance Publique - Hôpitaux de Paris, Clamart, France; Université Paris-Sud 11, Le Kremlin-Bicêtre, Villejuif, France; and.
authors.forename: Agnès
authors.initials: A
authors.lastname: Veyradier
authors.affiliation: Université Pierre et Marie Curie Sorbonne Universités, Université Paris 06, Paris, France; Centre Hospitalier Régional Saint-Antoine, Service d'Hématologie, AP-HP, Paris, France; Centre de Référence des Microangiopathies Thrombotiques, Hôpital Saint-Antoine, Assistance Publique - Hôpitaux de Paris, Paris, France; INSERM U1009, Institut Gustave Roussy, Villejuif, France.
authors.forename: Paul
authors.initials: P
authors.lastname: Coppo
mesh_terms: D051722:ADAM Proteins / Q000097:blood / Q000172:deficiency / Q000276:immunology; D000071120:ADAMTS13 Protein; D000328:Adult; D058846:Antibodies, Monoclonal, Murine-Derived / Q000008:administration & dosage*; D001323:Autoantibodies / Q000097:blood; D018890:Chemoprevention / Q000379:methods; D003430:Cross-Sectional Studies; D005260:Female; D006801:Humans; D007262:Infusions, Intravenous; D008297:Male; D011697:Purpura, Thrombotic Thrombocytopenic / Q000097:blood / Q000517:prevention & control*; D012074:Remission Induction; D012189:Retrospective Studies; D000069283:Rituximab; D055502:Secondary Prevention; D016896:Treatment Outcome
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D001323:Autoantibodies; D000069283:Rituximab; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human