Hemophagocytic Lymphohistiocytosis With Hyperacute Liver Failure Due to Ebstein-Barr Viremia

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Conference Proceeding

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Publication Title

American Journal of Respiratory Care and Critical Care Medicine


Hemophagocytic Lymphohistiocytosis (HLH) is an acute systemic syndrome due to severe immune activation resulting in multiple organ dysfunction and a high mortality. HLH can occur primarily or associated with several triggers including viral infections. We present a rare case of HLH and hyperacute liver failure (HALF) due to EBV infection. 50 y-o woman was admitted for fever and right orbit pain of 2-week duration. She had history of uncontrolled type 2 diabetes mellitus and hypertension. Antibiotics were started for periorbital cellulitis while CT scan and MRI of the orbits/brain showed non-specific right periorbital swelling without mass lesions or meningoencephalitis. She had leukocytosis and no neurological deficits. Her admission ferritin level was > 70,000 ng/ml prompting hematological workup including for HLH. Investigations demonstrated presence of markers of severe HLH syndrome. She was started on IV immuneglobulins empirically and symptoms improved. Bone marrow biopsy was performed on Day 5. On day 9, she was transferred to ICU for acute deterioration in mentation and respiratory distress. She was intubated and mechanically ventilated. Repeat neuroimaging showed no stroke, hemorrhage or mass effect. In addition to worsening parameters and bone marrow confirmation for HLH, her laboratory data suggested presence of fulminant liver failure including severe hyperammonemia and marked elevation of transaminases. Exhaustive diagnostic studies to determine the cause of HLH and liver failure suggested severe EBV viremia as the trigger for HLH and resultant acute liver failure (Table1). She could not receive biopsies of her liver or perioribital tissue to rule out lymphoma due to rapidly overwhelming vasodilatory shock and subsequent multiple organ failure.Her aggressive management included multiple vasoactive infusions, continuous venovenous hemofiltration, N-acetyl cysteine infusion, broad-spectrum antibiotics, blood product transfusions (pRBCs, plasma, platelets and cryoprecipitate), high dose corticosteroids, IV immuneglobulins, and rituximab. Despite these measures she passed away from refractory shock on ICU Day 7. HLH is a devastating clinical syndrome with high mortality. Markedly elevated ferritin or triglycerides should prompt investigations in the correct clinical context. Histiocyte Society criteria for HLH must be followed by identification of its trigger. HLH secondary to EBV has described in children and adults. Variants include those linked with genetic predisposition to HLH and those associated with lymphoma. Our case is unique being a previously unreported description of EBV viremia triggered secondary-HLH that resulted in hyperacute liver failure (acute liver failure < 7 days). Early diagnosis and intervention for liver failure must be a consideration in severe HLH.





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International Conference of the American Thoracic Society, May 19-24, 2023, Washington, DC