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Rituximab in a child with autoimmune thrombotic thrombocytopenic purpura refractory to plasma exchange
Authors:Parameswaran Narayanan  Aparna Jayaraman  Rashi S Rustagi  S Mahadevan  Sreejith Parameswaran
Institution:Department of Pediatrics, Jawaharlal Institute of Postgraduate Medical Education and Research, Dhanvantari Nagar, Pondicherry, India. narayanan@jipmer.net
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.
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