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: Parameswaran Narayanan, Aparna Jayaraman, Rashi S Rustagi, S Mahadevan, Sreejith Parameswaran
• Journal: International journal of hematology (Int J Hematol) Vol. 96 Issue 1 Pg. 122-4 (Jul 2012) ISSN: 1865-3774 [Electronic] Japan
• PMID: 22552883 (Publication Type: Case Reports, Journal Article)

Chemical References

• Antibodies, Monoclonal, Murine-Derived
• Immunologic Factors
• Rituximab
• ADAM Proteins
• ADAMTS13 Protein
• ADAMTS13 protein, human

Topics

• ADAM Proteins (immunology, metabolism)
• ADAMTS13 Protein
• Antibodies, Monoclonal, Murine-Derived (therapeutic use)
• Autoimmune Diseases (complications, diagnosis, therapy)
• Brain (pathology)
• Child
• Female
• Humans
• Immunologic Factors (therapeutic use)
• Infarction, Anterior Cerebral Artery (diagnosis, etiology)
• Magnetic Resonance Imaging
• Plasma Exchange
• Purpura, Thrombotic Thrombocytopenic (complications, diagnosis, therapy)
• Rituximab