SHM Converge Scientific Abstract ePoster Gallery-Evans Syndrome: Delayed Diagnosis Due to Cognitive Bias

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This case report describes a 65-year-old Spanish-speaking woman with a prior history of early-stage breast cancer who was under surveillance when she developed progressive cytopenias. Evans syndrome is a rare autoimmune disorder involving two or more immune-mediated blood cell deficiencies, most commonly autoimmune hemolytic anemia (AIHA) and immune thrombocytopenic purpura (ITP), with autoimmune neutropenia occurring less often. The report highlights anchoring bias, a cognitive error in which clinicians remain fixed on an initial diagnosis despite new evidence.<br /><br />The patient’s initial evaluation showed severe anemia, leukopenia, and mild thrombocytopenia. Laboratory findings supported an autoimmune hemolytic process, including elevated bilirubin, high reticulocyte count, positive direct antiglobulin test for IgG, and peripheral smear abnormalities. She was initially treated for AIHA with transfusion, IVIg, and rituximab. At a later visit, she was instead labeled as having NASH and portal hypertension. She continued to have recurrent anemia and worsening pancytopenia, and Evans syndrome was not recognized until a subsequent admission, when corticosteroids were started.<br /><br />Further imaging revealed a large right hilar mass, mediastinal lymphadenopathy, a new right adrenal nodule, and massive splenomegaly. Biopsy confirmed diffuse B-cell lymphoma, establishing Evans syndrome as a paraneoplastic manifestation of malignancy.<br /><br />The authors emphasize that diagnostic inaccuracies are often recognized late, especially in rare disorders where cognitive bias may delay reconsideration of the diagnosis. This case underscores the importance of maintaining suspicion for Evans syndrome in patients with multilineage cytopenias of autoimmune origin and of reassessing the differential diagnosis when the clinical course does not fit the initial impression.

Asset Subtitle

Murali Ranjani Behara

Meta Tag

Author List Dawood Findakly, Jue Wang, Murali Ranjani Behara

Category Clinical Vignettes

Concept Anchoring Bias

Concept Evans Syndrome

Concept Autoimmune Hemolytic Anemia

Concept Thrombocytopenia

Concept Delayed Diagnosis

Distinguished Non-Finalist

Presenter Organization Creighton University, Phoenix, AZ

Presenting Author Murali Ranjani Behara

Track Adult

Keywords

Evans syndrome

autoimmune hemolytic anemia

immune thrombocytopenic purpura

cytopenias

anchoring bias

paraneoplastic syndrome

diffuse B-cell lymphoma

pancytopenia

direct antiglobulin test

splenomegaly

Anchoring Bias

Evans Syndrome

Autoimmune Hemolytic Anemia

Thrombocytopenia

Delayed Diagnosis