A strange case of Evans syndrome

  • Manuel Monti | montimanuel@tiscali.it Emergency Department of AUSL Umbria 1, Assisi (PG), Italy.
  • Lucia Stefanecchia Emergency Department of AUSL Umbria 1, Assisi (PG), Italy.
  • Manolo Filippucci Emergency Department of AUSL Umbria 1, Assisi (PG), Italy.
  • Alessio Monti Engineering Department, Roma Tre University, Rome, Italy.
  • Giovanni Maria Vincentelli Emergency Department, Fatebenefratelli Isola Tiberina, Rome, Italy.
  • Francesco Borgognoni Emergency Department of AUSL Umbria 1, Assisi (PG); UO PS/118 AUSL Umbria 1, Assisi (PG), Italy.

Abstract

Evans syndrome is a rare autoimmune disease presenting hemolytic anemia, thrombocytopenia and/or neutropenia. It may be associated with other autoimmune or lymphoproliferative diseases. It can have an extremely serious disease course and, in rare cases, this can even be life-threatening. 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 report a case of Evans syndrome in a 54-year old woman admitted to the Emergency Department (ED) for asthenia. Etiopathogenic, clinical, therapeutic and evolutive aspects are discussed. In contrast to many cases described in the literature, our patient had a satisfactory response to corticoids. We also discuss how to make a specific diagnosis, even in a suburban ED with limited resources, in order to admit patients to the appropriate hospital department and allow the correct therapy to be started as early as possible.

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Published
2013-12-17
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Section
Case Reports
Keywords:
Evans syndrome, autoimmune hemolytic anemia, idiopathic thrombocytopenic purpura, blood cells, immune system diseases.
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How to Cite
Monti, M., Stefanecchia, L., Filippucci, M., Monti, A., Vincentelli, G. M., & Borgognoni, F. (2013). A strange case of Evans syndrome. Italian Journal of Medicine, 7(4), 305-309. https://doi.org/10.4081/itjm.2013.305