From Hemolysis to Lupus: A Case of Evans Syndrome Revealing Systemic Autoimmunity

Abstract

Evans syndrome (ES), the coexistence of autoimmune hemolytic anemia and immune thrombocytopenia, can unmask systemic autoimmune disease. We report a 30-year-old woman who presented with fatigue, jaundice, pallor, and mucocutaneous bleeding. Laboratory evaluation demonstrated severe Coombs-positive hemolytic anemia (hemoglobin 5.8 g/dL, reticulocytosis, high LDH, undetectable haptoglobin) and marked thrombocytopenia, whereas immunologic testing revealed high-titer ANA, anti-dsDNA positivity, and low complement levels; infections, thrombotic microangiopathy, and malignancy were excluded. She was stabilized with transfusion and treated with high-dose corticosteroids and intravenous immunoglobulin, achieving a partial response. Persistent cytopenias prompted rituximab, after which blood counts normalized and hemolysis resolved without major infectious or thrombotic events. At 6 months, she remained in remission on maintenance therapy. This case emphasizes that ES can be the initial manifestation of systemic lupus erythematosus and supports early autoimmune evaluation and tailored immunosuppression to address both cytopenias and underlying disease.