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Effectiveness of cyclosporine and mycophenolate mofetil in a child with refractory evans syndrome

Piero Farruggia, Alessandra Macaluso, Serena Tropia, Selene Genova, Olivia Paolicchi, Floriana Di Marco, Paolo D'Angelo

Authors information
  • Piero Farruggia
    Pediatric Hematology and Oncology Unit, "G. Di Cristina" Children's Hospital, ARNAS Palermo, Italy. pfarruggia@libero.it
  • Alessandra Macaluso
    Department of Pediatrics, “G. Di Cristina” Children’s Hospital, University of Palermo, Palermo, Italy., Italy.
  • Serena Tropia
    Pediatric Hematology and Oncology Unit, "G. Di Cristina" Children's Hospital, ARNAS Palermo, Italy.
  • Selene Genova
    Department of Pediatrics, “G. Di Cristina” Children’s Hospital, University of Palermo, Palermo, Italy., Italy.
  • Olivia Paolicchi
    Department of Pediatric Hematology and Oncology, A.O.U. Meyer Children Hospital, Florence, Italy., Italy.
  • Floriana Di Marco
    Pediatric Hematology and Oncology Unit, "G. Di Cristina" Children's Hospital, ARNAS Palermo, Italy.
  • Paolo D'Angelo
    Pediatric Hematology and Oncology Unit, "G. Di Cristina" Children's Hospital, ARNAS Palermo, Italy.

Abstract


Evans Syndrome is a rare autoimmune disease consisting of hemolytic anemia, thrombocytopenia and/or neutropenia. It may be associated with other autoimmune or lymphoproliferative diseases. Its course can be extremely serious and, rarely, even life-threatening; thus it represents a excellent treatment challenge for the pediatric hematologist. First line treatment consists of steroids and/or immunoglobulin; further therapy with rituximab, vincristine, cyclophosphamide and other immunosuppressive drugs can be considered in unresponsive patients. We describe a baby with refractory Evans Syndrome that was cured by prolonged administration of mycophenolate mofetil and remained disease-free for 4 years after the discontinuation of treatment.

Keywords


Evans’ syndrome, Cyclosporine, Mycophenolate mofetil, Treatment, Outcome

Full Text





Submitted: 2011-03-24 23:02:10
Published: 2011-05-16 09:07:57
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Pediatric Reports [eISSN 2036-7503] is an Open Access, peer-reviewed journal published by PAGEPress, Pavia, Italy. All credits and honors to PKP for their OJS.

 
 
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