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

  • Piero Farruggia | pfarruggia@libero.it Pediatric Hematology and Oncology Unit, "G. Di Cristina" Children's Hospital, ARNAS Palermo, Italy.
  • 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.

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Author Biographies

Piero Farruggia, Pediatric Hematology and Oncology Unit, "G. Di Cristina" Children's Hospital, ARNAS Palermo

Pediatric Hematology and Oncology Unit, "G. Di Cristina" Children's Hospital, A.R.N.A.S. Ospedali Civico, Di Cristina e Benfratelli, Palermo, Italy.

Pediatrician

Alessandra Macaluso, Department of Pediatrics, “G. Di Cristina” Children’s Hospital, University of Palermo, Palermo, Italy.

Department of Pediatrics, “G. Di Cristina” Children’s Hospital, University of Palermo, Palermo, Italy.

Attending to Pediatrics Postgraduate School

Serena Tropia, Pediatric Hematology and Oncology Unit, "G. Di Cristina" Children's Hospital, ARNAS Palermo

Pediatric Hematology and Oncology Unit, "G. Di Cristina" Children's Hospital, A.R.N.A.S. Ospedali Civico, Di Cristina e Benfratelli, Palermo, Italy.

Pediatrician

Selene Genova, Department of Pediatrics, “G. Di Cristina” Children’s Hospital, University of Palermo, Palermo, Italy.

Department of Pediatrics, “G. Di Cristina” Children’s Hospital, University of Palermo, Palermo, Italy.

Attending to Pediatrics Postgraduate School

Olivia Paolicchi, Department of Pediatric Hematology and Oncology, A.O.U. Meyer Children Hospital, Florence, Italy.

Department of Pediatric Hematology and Oncology, A.O.U. Meyer Children Hospital, Florence, Italy.

Attending to Graduate School

Floriana Di Marco, Pediatric Hematology and Oncology Unit, "G. Di Cristina" Children's Hospital, ARNAS Palermo

Pediatric Hematology and Oncology Unit, "G. Di Cristina" Children's Hospital, A.R.N.A.S. Ospedali Civico, Di Cristina e Benfratelli, Palermo, Italy.

Pediatrician (Fellowship)

Paolo D'Angelo, Pediatric Hematology and Oncology Unit, "G. Di Cristina" Children's Hospital, ARNAS Palermo

Pediatric Hematology and Oncology Unit, "G. Di Cristina" Children's Hospital, A.R.N.A.S. Ospedali Civico, Di Cristina e Benfratelli, Palermo, Italy.

Head of staff

Published
2011-05-16
Info
Issue
Section
Case Reports & Letters
Keywords:
Evans’ syndrome, Cyclosporine, Mycophenolate mofetil, Treatment, Outcome
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How to Cite
Farruggia, P., Macaluso, A., Tropia, S., Genova, S., Paolicchi, O., Di Marco, F., & D’Angelo, P. (2011). Effectiveness of cyclosporine and mycophenolate mofetil in a child with refractory evans syndrome. Pediatric Reports, 3(2), e15. https://doi.org/10.4081/pr.2011.e15