Successful treatment with rituximab for acute refractory thrombotic thrombocytopenic purpura related to acquired ADAMTS13 deficiency: a pediatric report and literature review

Pediatric Critical Care Medicine : a Journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies
Jérôme HarambatOlivier Brissaud

Abstract

To report the case of a child with severe autoimmune thrombotic thrombocytopenic purpura (TTP) resistant to plasma exchange and steroids who was successfully treated with rituximab. Case report and review of the literature on pediatric acquired TTP. The report was approved by an independent local ethics committee. Pediatric intensive care unit in a tertiary care children's hospital. A 10-yr-old boy was referred to the emergency unit with fever, vomiting, confusion, hemolytic anemia, thrombocytopenia, and mild acute renal failure. An atypical hemolytic uremic syndrome was suspected, and plasma exchange was started urgently. The patient was refractory to plasma therapy and presented critical complications. After a diagnosis of acquired TTP attributable to anti-ADAMTS13 autoantibodies had been made, he was treated with rituximab, which resulted in a stable clinical remission. Rituximab therapy. Clinical remission. TTP is a rare but life-threatening condition in children that is characterized by hemolytic anemia, thrombocytopenia, and signs of ischemic organ dysfunction. If renal involvement is present, TTP may be misdiagnosed as hemolytic uremic syndrome, but reliable screening for ADAMTS13 activity and anti-ADAMTS autoantibodies ...Continue Reading

References

Feb 18, 2003·British Journal of Haematology·Sarah L AllfordUNKNOWN Haemostasis and Thrombosis Task Force, British Committee for Standards in Haematology
Apr 8, 2003·Journal of Pediatric Hematology/oncology·Terzah M HortonDonald H Mahoney
Jan 17, 2004·Seminars in Hematology·Reinhard SchneppenheimTobias Obser
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Mar 27, 2007·The Journal of Pediatrics·Lisa B GiulinoUNKNOWN Pediatric and Platelet Immunology Committees of the TMH Clinical Trial Network
Mar 27, 2007·The Journal of Pediatrics·Moussa El-HallakRobert P Sundel
Apr 28, 2009·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Marie-Camille ChaumaisGeorges Deschênes
Apr 29, 2009·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Jumana H AlbaramkiStephen I Alexander

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Citations

Dec 17, 2010·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Sybille PelrasJérôme Harambat
May 4, 2012·International Journal of Hematology·Parameswaran NarayananSreejith Parameswaran
Mar 12, 2013·Reumatología clinica·Raimundo Garcia BoyeroMiguel Angel Belmonte Serrano
Nov 20, 2013·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Damien NooneChristoph Licht
May 4, 2013·Pediatric Clinics of North America·Joshua Cappell, Steven G Kernie
Jan 24, 2012·La Presse médicale·Paul Coppo, Agnès Veyradier
Dec 19, 2014·Pediatrics International : Official Journal of the Japan Pediatric Society·Senthilkumar SankararamanHassan Ibrahim
Feb 21, 2013·Current Opinion in Pediatrics·Chantal LoiratAgnès Veyradier

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