Case Presentation: 81-year-old man initially presented to his primary care physician for a persistent cough of three weeks associated with new onset shortness of breath when climbing stairs. He had a normal chest X-Ray, but also a mild anemia. Initial work-up was targeted at evaluation for a gastrointestinal bleed. He underwent a CT abdomen/pelvis, upper endoscopy and lower endoscopy all of which were normal. A few weeks later at his primary care follow-up visit, his hemoglobin was persistently low and he was sent to the Emergency Room. He complained of a persistent, non-productive cough, shortness of breath, and a single episode of night sweats in the previous week. He was normotensive, afebrile, and tachycardic with a heart rate of 121. He had a small left-sided post-auricular lymph node without splenomegaly. His exam was otherwise normal. Hgb was 6.3, and Reticulocyte Index was 0.03. Platelets reached a low of 101. There was no evidence of liver enzyme derangements or iron deficiency. His hemoglobin responded appropriately to 2 units of packed red blood cells. Gastroenterology was initially consulted for presumed occult GI bleed and was considering a capsule endoscopy. However, direct antiglobulin testing (DAT) subsequently was positive, followed by a positive EBV PCR. The patient was diagnosed with EBV associated Evans syndrome and started on IVIG and steroids. On hospital follow-up 1.5 months later, hemoglobin had increased to 11.6.
Discussion: Evans Syndrome is a rare condition with an incidence of 1.8 per million person-years. It is characterized by positive DAT autoimmune hemolytic anemia and immune thrombocytopenia. Anemia and thrombocytopenia can happen either simultaneously or sequentially. Immune neutropenia is also possible. It can be idiopathic or caused by infections, autoimmune diseases, lymphoproliferative diseases, and CVID. Our patient developed Evans syndrome secondary to EBV infection which is also uncommon in the elderly population. First line treatment for Evans Syndrome includes glucocorticoids and IVIG. Second line therapy includes rituximab.
Conclusions: Our case is important to Hospital Medicine because it both describes a rare condition and emphasizes the dangers of anchoring bias. In an elderly man, a gastrointestinal bleed is fairly common, whereas infectious mononucleosis leading to Evans Syndrome is rare. Consequently, this patient over the course of several weeks underwent multiple invasive tests without obtaining a diagnosis. He was then being considered for further GI work-up including a capsule endoscopy until DAT sent on admission returned positive. This demonstrates the need for hospitalists to reconsider a broad differential on admission especially when patients already have completed a basic work-up for common entities. Furthermore, consideration should be given to atypical presentations of common illnesses in different patient demographics. In young adult patients, infectious mononucleosis presents with fever, pharyngitis, and lymphadenopathy; however, in older patients, infectious mononucleosis is less likely to present with typical symptoms.