Case Presentation: A 33 year-old Caucasian female with history of 1 spontaneous abortion presented with periorbital edema, dark urine, arthralgias and fevers (Tmax 102) for 2 weeks. The patient reported many environmental exposures as a native of Brisbane, Australia such as outbreaks of Ross River Fever, encounters with flying foxes (reservoirs for Lyssa, Hendra viruses), a layover in Hong Kong, and a toddler with a febrile illness. She sought outpatient care and initial laboratory evaluation revealed WBC 2.52K/uL, platelets to 105K/uL and AST/ALT 88/70 ALP 117 U/L. She was prescribed a course of Amoxicillin/Clavulanate without improvement. A week later, she developed pleuritic chest pain and was found to have a small pericardial effusion on outpatient echocardiogram and referred to the Emergency Department.Triage vitals were normal. Her exam was significant for marked periorbital edema, cervical lymphadenopathy and pain in bilateral wrists without effusion. Labs showed recovering blood counts (WBC 9.3K/uL Hgb 13.1g/dL and Plt 157 K/uL), unremarkable urinalysis, microalbumin/creatinine ratio and CK, but worsening LFTs with AST 505 ALT 578 ALP 788 U/L. She was admitted for further work-up with rheumatology and infectious disease input. During hospitalization, the patient developed additional symptoms of drenching night sweats and sore throat.
Repeat echocardiography revealed a trace pericardial effusion while abdominal ultrasound showed a liver with normal contour and echotexture. C3/C4 levels, beta-2 glycoprotein, cardiolipin, Ro/La, lupus anticoagulant, histone, centromere, and mitochondrial antibodies all returned negative, as did respiratory viral panel, HIV, hepatitis serologies, thick and thin smears, Lyme, Anaplasma and Babesia serologies, Ross River fever, Dengue and Chikungunya. ANA titer and dsDNA Ab were < 1:40 and 9 respectively, but EBV viral capsid IgM returned positive consistent with acute mononucleosis.
Discussion: The triad of acute Epstein-Barr viral infection (EBV) involves high fevers, lymphadenopathy, and pharyngitis, all present in our patient. However, she also exhibited less typical features of the disease. Patients may have periorbital edema, “Hoagland’s sign,” caused by viral replication obstructing lymphatic drainage of the nasopharynx. Tonsillar B-cells may be similarly affected, spurring secretion of polyclonal antibodies (including heterophile and non-specific autoantibodies). While this process is normally accompanied by leukocytosis with atypical lymphocytes, our patient presented initially with leukopenia. Further, her degree of transaminitis with levels >10x normal is usually restricted to the immunocompromised. Finally, EBV may also cause transient myo- or pericarditis as noted sparingly in literature.
The mainstay of treatment is supportive, although glucocorticoids and acyclovir are used in cases of laryngeal edema, liver failure, or hemolytic anemia and thrombocytopenia. These agents have not been proven to reduce the length or severity of illness.
Conclusions: While the stereotyped triad of acute EBV is well known, atypical presentations provide diagnostic challenges, have the potential for life threatening complications, and warrant further evaluation. After several days of inpatient monitoring, our patient was discharged home with supportive therapy.