Unmasking Evans Syndrome: A Case of New Onset Anemia and Thrombocytopenia in a Pregnant Patient With Systemic Lupus Erythematosus.

Abstract

Evans Syndrome (ES) is an immune-mediated disorder defined by the presence of two or more autoimmune cytopenias, typically autoimmune hemolytic anemia (AIHA) and immune thrombocytopenic purpura (ITP), with or without associated neutropenia. The disease can be classified as primary (i.e., idiopathic) or secondary to other underlying conditions, such as systemic lupus erythematosus (SLE), lymphoproliferative disorders, infections, and immunodeficiency states. We present a case of ES in a 21-year-old pregnant woman (G1P0, 19 weeks and 2 days gestation) secondary to SLE. The patient was treated with high-dose steroids and intravenous immunoglobulin therapy (IVIG), resulting in marked improvement in her cell counts. Following delivery, she was diagnosed with SLE and began treatment with rituximab and hydroxychloroquine, leading to remission of her symptoms and normalization of her anemia and thrombocytopenia. The varied presentations and associated conditions make ES a challenging diagnostic conundrum. Differentiating between primary and secondary ES is crucial for prognosis and management.