Introduction
Epstein-Barr virus (EBV) is a herpes virus that commonly causes infectious mononucleosis (IM) and linked to different hematological conditions. Here we present a case of EBV-triggered Hemolytic Uremic Syndrome (HUS) with pulmonary involvement.
Case Presentation
A 20-year-old male presented with fever, thrombocytopenia, and splenomegaly. Acute EBV serology was positive. Creatinine and hemoglobin were normal. He was diagnosed with IM. platelet count improved within 3 weeks. 4 weeks later, he returned with severe hemoptysis. Hgb 6.8g/dL, platelet 133,000/uL, lactate dehydrogenase 969u/L, creatinine 21mg/dL, and schistocytes on peripheral smear. Chest computed tomography showed bilateral opacities consistent with diffuse alveolar hemorrhage (DAH). Emergent hemodialysis and plasmapheresis were started. Infectious work up was negative. Autoimmune work up was also negative (anti-neutrophil cytoplasmic, anti-basement membrane antibodies, ANA). Aadamts13 activity was 62% (normal ~66%) ruling out thrombotic thrombocytopenic purpura (TTP). Kidney biopsy revealed thrombotic microangiopathic process. The patient was eventually diagnosed with HUS and treated with Eculizumab. 4 months later his renal function has partially recovered and no longer needs hemodialysis.
Discussion
HUS is a rare entity that is known to be triggered by different underlying pathologies. However, its link to EBV remains unclear. Literature review has revealed only two cases of EBV-triggered HUS, even though almost 90-95% of adults are EBV-seropositive. What unique about our case is the patient initially presented with documented IM, and HUS happened a month later. This raises the theory that HUS could be a sequela of the infection, rather than an effect of acute viral phase and this is the first case to report such correlation. The other unique thing is pulmonary involvement in HUS. With consultation with pulmonary service, we believe our patient had DAH based on clinical and radiographic findings. To our knowledge this is the first case to show this association.
Conclusion
EBV is a common virus with high seropositivity among world’s population. Its link to HUS remains unclear and needs more investigation. Providers should recognize HUS as a complication of EBV infection, either in the acute phase or as a sequela. Adolescents are at higher risk for such complication since IM is common in this population.
