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Liver recovery in a child with hemophagocytic lymphohistiocytosis-induced acute liver failure


 Introduction: Hemophagocytic lymphohistiocytosis is a rare condition with dysregulated multi-organ inflammation that may cause acute liver failure. It often presents with non-specific clinical features and can be difficult to diagnose.

 Case Report: We present the case of a 2-year-old girl with lethargy, encephalopathy, poor oral intake, vomiting, and jaundice. Her labs indicated acute liver failure with additional findings of bicytopenia and elevated ferritin. Given high concern for hemophagocytic lymphohistiocytosis, we promptly consulted the hematology/oncology, transplant hepatology, and pharmacy services. Bone marrow biopsy initially showed no evidence of hemophagocytosis. Given our high clinical index of suspicion for hemophagocytic lymphohistiocytosis and its high mortality and morbidity, we initiated treatment with high dose dexamethasone, etoposide, and the recently approved biologic drug emapalumab, a monoclonal antibody against interferon gamma, despite not meeting full HLH-2004 diagnostic criteria. One day after treatment initiation, the final review of the bone marrow biopsy showed evidence of hemophagocytosis. Ultimately, with implementation of multidisciplinary rounds, close neurologic examinations, aggressive management of evolving hyperammonemia and cerebral edema, and early initiation of treatment, our patient achieved full liver recovery.

 Conclusion: This patient’s presentation emphasizes the importance of having a broad differential when a patient presents with liver failure and cytopenias. Our management of this patient showcases the importance of expedient, multidisciplinary management for a complex critically ill pediatric patient. The patient’s survival and complete liver recovery with the treatment protocol given suggests emapalumab should be studied in future clinical trials as an important adjunctive treatment for patients with hemophagocytic lymphohistiocytosis with acute liver failure.
