title: Rituximab in a child with autoimmune thrombotic thrombocytopenic purpura refractory to plasma exchange.
abstract: A nine-year-old girl presented with headache, purpura and mild left hemiparesis. Laboratory evaluation revealed thrombotic microangiopathy with ADAMTS13 deficiency, with auto-antibodies to ADAMTS13. She was treated with plasma exchange and steroids, following which she improved transiently, relapsing within 2 months. The relapse was refractory to conventional therapy and rituximab was tried. She had good response to rituximab and has been in remission for the past 12 months. Rituximab may be a promising option for children with acquired TTP refractory to plasma exchange and steroids.
authors.affiliation: Department of Pediatrics, Jawaharlal Institute of Postgraduate Medical Education and Research, Dhanvantari Nagar, Pondicherry, India. narayanan@jipmer.net
authors.forename: Parameswaran
authors.initials: P
authors.lastname: Narayanan
authors.forename: Aparna
authors.initials: A
authors.lastname: Jayaraman
authors.forename: Rashi S
authors.initials: RS
authors.lastname: Rustagi
authors.forename: S
authors.initials: S
authors.lastname: Mahadevan
authors.forename: Sreejith
authors.initials: S
authors.lastname: Parameswaran
mesh_terms: D051722:ADAM Proteins / Q000276:immunology / Q000378:metabolism; D000071120:ADAMTS13 Protein; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use*; D001327:Autoimmune Diseases / Q000150:complications / Q000175:diagnosis / Q000628:therapy*; D001921:Brain / Q000473:pathology; D002648:Child; D005260:Female; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use*; D020243:Infarction, Anterior Cerebral Artery / Q000175:diagnosis / Q000209:etiology; D008279:Magnetic Resonance Imaging; D010951:Plasma Exchange; D011697:Purpura, Thrombotic Thrombocytopenic / Q000150:complications / Q000175:diagnosis / Q000628:therapy*; D000069283:Rituximab
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D007155:Immunologic Factors; D000069283:Rituximab; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human