S2758 A Case of Hemophagocytic Lymphohistiocytosis Presenting With Acute Liver Injury

Abstract

Introduction: Hemophagocytic lymphohistiocytosis (HLH) is a syndrome with high rates of mortality marked by inappropriate immune activation of T-cells and macrophages that manifests with multi-organ dysfunction. HLH is primarily observed among pediatric patients and is exceedingly rare in adults. Here we present a case of acute liver injury with centrizonal hepatocellular necrosis on liver biopsy, secondary to HLH in an adult male. Case Description/Methods: Our patient is a 33-year-old white male resident of Guam with extensive binge drinking history who presented with two weeks of dry cough, generalized malaise, and fevers. Initial serologic workup was notable for anemia, leukopenia, elevated inflammatory markers, and elevated liver transaminases 400-500 U/L. Given his nonspecific symptoms and lab findings, there was moderate clinical concern for HLH. CT of the abdomen revealed hepatosplenomegaly with multifocal areas of nodular hyperenhancement of the liver. An MRI of the liver demonstrated multiple hypervascular nodules most consistent with a nonspecific inflammatory process. PET scan showed diffuse hypermetabolism throughout the liver consistent with inflammation. A liver biopsy demonstrated centrizonal hepatocellular necrosis. Despite biopsy results, clinical concern remained high for HLH and he was transferred to a higher level of care for further workup by a multidisciplinary team including Hepatology. Extensive rheumatologic and infectious workup, including hepatidities, were negative. At this time a clinical diagnosis of HLH was made based off HLH-2004 diagnostic criteria fever > 38.5°C, hypertriglyceridemia > 265 mg/dL, ferritin > 500ng/mL, elevated sCD25, and splenomegaly. Treatment was initiated with dexamethasone and IVIG with near resolution of his acute liver injury within two months of treatment onset. Discussion: HLH is an exceptionally rare syndrome among adults (one per 800,000 annually) that presents with many nonspecific signs and symptoms. This causes diagnostic difficulty making it crucial to maintain a broad differential to aid in proper diagnosis and to prevent the delay in potentially lifesaving treatment. Elevated liver transaminases are common among HLH patients, however the pattern of necrosis seen on biopsy in this patient is unique compared to current literature. This case represents the importance of maintaining a broad differential in patients with diffuse inflammatory symptoms in the setting of nonspecific liver inflammation, as seen in this exceedingly rare case of HLH.