A woman with Sjogren's syndrome and a new diagnosis of Evans syndrome: a case report.

Abstract

Evans syndrome (ES) is a rare disease characterized by the simultaneous or sequential development of autoimmune hemolytic anemia (AIHA) with immune thrombocytopenia (ITP), and less frequently autoimmune neutropenia. ES can be primary or secondary to another disease. Isolated immune cytopenias have been reported with Sjogren's syndrome (SS) in the past, but the association with ES has very rarely been reported. We present a patient with a known history of SS who presented with mild bleeding symptoms, such as heavier menses and new ecchymoses. She was found to have a very low haptoglobin, spherocytes on peripheral smear, positive direct antiglobulin test, and positive eluate testing. The findings were consistent with severe ITP and AIHA, leading to a diagnosis of secondary ES. She was treated with high-dose steroids for 4 days concurrent with 2 days of intravenous immunoglobulin (IVIG) with marked improvement in her hematological function. She was discharged on a steroid taper and remained in remission at follow-up visits. Although ES is a rare presentation of autoimmune disease, it is associated with high mortality and necessitates prompt clinical identification and appropriate therapy selection. Further research is necessary to understand the associated clinical characteristics, determine prognosis, and provide management recommendations.