title: Rituximab in autoimmune thrombotic thrombocytopenic purpura: A success story.
abstract: Despite a significant improvement of thrombotic thrombocytopenic purpura (TTP) prognosis since the use of plasma exchange, morbidity and mortality remained significant because of poor response to standard treatment or exacerbations and relapses. Rituximab, a chimeric monoclonal antibody directed against the B-lymphocyte CD20 antigen, has shown a particular interest in this indication. Recent studies also reported strong evidence for its efficiency in the prevention of relapses. This review addresses these recent progresses and still opened questions in this topic: should rituximab be proposed in all patients at the acute phase? Should all patients benefit from a preemptive treatment? Is the infectious risk acceptable in this context?
authors: Service de médecine interne, CHI, Créteil, France; Centre de Référence des Microangiopathies Thrombotiques, AP-HP, Paris, France.
authors: Antoine
authors: A
authors: Froissart
authors: Centre de Référence des Microangiopathies Thrombotiques, AP-HP, Paris, France; Service d'hématologie biologique, Hôpital Lariboisière, Paris, France; Université Paris Diderot, Sorbonne Paris Cité, Paris, France.
authors: Agnès
authors: A
authors: Veyradier
authors: Service de Médecine Interne, Hôpital la Pitié-Salpétrière, Paris, France.
authors: Miguel
authors: M
authors: Hié
authors: Centre de Référence des Microangiopathies Thrombotiques, AP-HP, Paris, France; Service de médecine interne, CHU Charles Nicolle, Rouen, France.
authors: Ygal
authors: Y
authors: Benhamou
authors: Centre de Référence des Microangiopathies Thrombotiques, AP-HP, Paris, France; Service d'hématologie, Hôpital Saint Antoine, Paris, France; Inserm U1009, Institut Gustave Roussy, Villejuif, France. Electronic address: paul.coppo@aphp.fr.
authors: Paul
authors: P
authors: Coppo
mesh_terms: D051722:ADAM Proteins / Q000172:deficiency / Q000378:metabolism*; D000071120:ADAMTS13 Protein; D018501:Antirheumatic Agents / Q000008:administration & dosage* / Q000009:adverse effects; D001402:B-Lymphocytes / Q000187:drug effects; D018450:Disease Progression; D006801:Humans; D007167:Immunotherapy / Q000379:methods; D010951:Plasma Exchange; D011697:Purpura, Thrombotic Thrombocytopenic / Q000628:therapy*; D012008:Recurrence; D000069283:Rituximab / Q000008:administration & dosage* / Q000009:adverse effects
chemical_list: D018501:Antirheumatic Agents; D000069283:Rituximab; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human
keywords: ADAMTS13; Immunotherapy; Rituximab; Thrombotic thrombocytopenic purpura