Case Presentation: A 65-year-old woman with a history of early-stage breast cancer—was treated six years prior. She was subsequently admitted multiple times over the following years for pancytopenia, which was thought to be multifactorial—due to nonalcoholic steatohepatitis and auto-immune hemolytic anemia (AIHA). Therefore, she received a supportive blood transfusion, intravenous immunoglobulins, and rituximab for suspected AIHA. Even though hematology was consulted during these events, no further workup was pursued and she was advised to follow up in the outpatient clinic after stabilization of her condition. Eventually, she presented to the ED with an episode of fatigue, nausea, and vomiting. Her physical exam was notable for pallor and mild diffuse abdominal tenderness. Her hemoglobin was 5.4 g/dl, platelets were 63,000/μL, total bilirubin was 2.1 μg/dl, absolute reticulocytes count was 7,4480/mm3, haptoglobin 64mg/dl, lactate dehydrogenase was 172 U/L, and IgG-positive direct antiglobulin test. The peripheral smear showed rare schistocytes suggestive of autoimmune process. CT scan revealed a large right hilar mass with multiple mediastinal lymph nodes (largest 4cm), new right adrenal nodule, and massive splenomegaly and she was admitted for obstructive pneumonia. Hematology was consulted for pancytopenia, and she was diagnosed with Evans syndrome, presenting as a paraneoplastic syndrome. An endobronchial right hilar mass biopsy was consistent with diffuse B-cell lymphoma, non-germinal center type with FISH negative for BCL2/BCL6/MYC rearrangements. In the meantime, she was started on high-dose steroids after which her hematologic parameters improved. Thereafter, the patient was started on rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) chemotherapy regimen.
Discussion: Evans syndrome is a rare autoimmune disorder characterized by synchronous or consecutive multi-lineage cytopenia, particularly AIHA, immune thrombocytopenic purpura (ITP), and infrequently, autoimmune neutropenia (AIN). Upon our patient’s evaluation by prior physicians, the anemia and thrombocytopenia were attributed to her NASH. When the patient eventually presented to our facility with an acute episode of abdominal pain, the workup for her anemia showed hemolysis secondary to ES. Moreover, upon further workup, the patient was eventually diagnosed with DLBCL causing secondary ES.Anchoring bias is the cognitive bias of fixating on one’s original impression despite evolving and disproving evidence.
Conclusions: Diagnostic inaccuracies are under-recognized, and often identified late in the disease process. Cognitive bias makes it even more challenging, especially when evaluating rare diseases. This report helps illustrates Anchoring Bias, a type of Cognitive bias. Physicians should maintain a high index of suspicion for Evans syndrome when evaluating patients with multi-lineage cytopenia of an autoimmune nature.