A strange case of Evans syndrome

• Published in: Italian Journal of Medicine
• Main Authors: Manuel Monti, Lucia Stefanecchia, Manolo Filippucci, Alessio Monti, Giovanni Maria Vincentelli, Francesco Borgognoni
• Format: Article
• Language: English
• Published: PAGEPress Publications 2013-12-01
• Subjects: Evans syndrome, autoimmune hemolytic anemia, idiopathic thrombocytopenic purpura, blood cells, immune system diseases.
• Online Access: http://www.italjmed.org/index.php/ijm/article/view/95
• ISSN: 1877-9344; 1877-9352

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.