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

TL;DR

A case of Evans syndrome revealed underlying systemic lupus, highlighting the importance of early diagnosis and targeted treatment for better outcomes.

Contribution

This case highlights that Evans syndrome can be the first sign of systemic lupus and emphasizes the need for early autoimmune evaluation.

Findings

The patient showed Coombs-positive hemolytic anemia and immune thrombocytopenia with high ANA and anti-dsDNA levels.

Treatment with corticosteroids, IV immunoglobulin, and rituximab led to remission without major complications.

The case supports the idea that Evans syndrome can be the initial manifestation of systemic lupus erythematosus.

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.

Evans syndrome can be the presenting feature of systemic lupus erythematosus. Prompt recognition of Coombs‐positive hemolytic anemia with immune thrombocytopenia, exclusion of alternative causes (infection, malignancy, and TTP, which can be Coombs‐negative), and early immunosuppression tailored to both the cytopenias and underlying autoimmunity, with rituximab for refractory cases, improve outcomes and warrant long‐term follow‐up.