Primary Evans syndrome (ES) is defined by the concurrent or sequential 
occurrence of immune thrombocytopenia and autoimmune hemolytic anemia in the 
absence of an underlying etiology. The syndrome is characterized by a chronic, 
relapsing, and potentially fatal course requiring long-term immunosuppressive 
therapy. Treatment of ES is hardly evidence-based. Corticosteroids are the 
mainstay of therapy. Rituximab has emerged as the most widely used second-line 
treatment, as it can safely achieve high response rates and postpone 
splenectomy. An increasing number of new genetic defects involving critical 
pathways of immune regulation identify specific disorders, which explain cases 
of ES previously reported as "idiopathic".