A Rare and Fatal Co-Occurrence of Evans Syndrome and Autoimmune Hepatitis/Primary Biliary Cirrhosis Overlap Syndrome

Evans syndrome (ES) is the concurrent occurrence of two or more lines of immune-mediated cytopenias commonly involving autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP). It can be an idiopathic condition or may be associated with lymphoproliferative and autoimmune disorders. Here we report a case of a person with a history of Autoimmune Hepatitis (AIH) and Primary Biliary Cirrhosis (PBC) Overlap Syndrome, on chronic steroids, who presented with thrombocytopenia and autoimmune hemolytic anemia and diagnosed with Evans syndrome. This case brings forth the difficulty in diagnosing ES, especially in patients with liver disease who can have overlapping laboratory abnormalities and the poor prognosis associated with the condition, despite treatment with IVIg and immunomodulatory agents, like rituximab.