SHM Converge Scientific Abstract ePoster Gallery-Evans Syndrome: An Unusual Case of Thrombocytopenia

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This case report describes a 24-year-old Caucasian woman with a family history of autoimmune disease who presented with three weeks of easy bruising, petechiae, and fatigue. Initial labs showed severe thrombocytopenia and anemia. Further testing revealed elevated LDH and reticulocytes, low haptoglobin, indirect bilirubinemia, and a positive direct antiglobulin test for both C3 and IgG, confirming warm autoimmune hemolytic anemia. After other causes were ruled out, she was diagnosed with Evans syndrome, a rare autoimmune disorder involving the combination of warm autoimmune hemolytic anemia and immune thrombocytopenia, sometimes with neutropenia.<br /><br />Her platelet count remained critically low despite methylprednisone and platelet transfusions, which produced only a brief response. She was started on high-dose prednisone and initially remained stable, but by hospital day 5 her platelet count fell to 5,000. She then received IVIG, which led to a strong platelet recovery to 51,000, and she was discharged on prednisone with hematology follow-up. Post-discharge labs showed improving hemoglobin and platelet counts.<br /><br />The report emphasizes that Evans syndrome is a diagnosis of exclusion and can be difficult to treat because of frequent relapses and higher mortality than isolated autoimmune cytopenias. First-line therapy is corticosteroids, but additional treatments such as IVIG, rituximab, and splenectomy may be required depending on response. In this patient, platelet transfusions were ineffective, IVIG was effective for acute thrombocytopenia, and later rituximab and splenectomy were used for ongoing disease control. The case highlights the need for better treatment guidelines for Evans syndrome.

Asset Subtitle

Naisarg B. Vanani

Meta Tag

Author List Alexandra A. Ahrens, Naisarg B. Vanani, Pinky Jha

Category Clinical Vignettes

Concept Evans Syndrome

Concept Warm Autoimmune Hemolytic Anemia

Concept Immune Thrombocytopenia

Concept Hemolysis

Concept Direct Antiglobulin Test

Distinguished Non-Finalist

Presenter Organization Medical College of Wisconsin

Presenting Author Naisarg B. Vanani

Track Adult

Keywords

Evans syndrome

warm autoimmune hemolytic anemia

immune thrombocytopenia

thrombocytopenia

direct antiglobulin test

intravenous immunoglobulin

corticosteroids

rituximab

splenectomy

autoimmune cytopenia

Evans Syndrome

Warm Autoimmune Hemolytic Anemia

Immune Thrombocytopenia

Hemolysis

Direct Antiglobulin Test