S2614 An Unconventional Presentation of Hemophagocytic Lymphohistiocytosis

Abstract

Introduction: Hemophagocytic Lymphohistiocytosis (HLH) characterized as a cytokine storm secondary to a dysregulated immune state predominantly affects the pediatric population but, reports of adult cases are increasing. The cytokine storm mediated by natural killer cells, macrophages and cytotoxic lymphocytes can be triggered by a genetic variation, infection, malignancy, or rheumatologic disease. This case discusses an unconventional presentation of HLH. Case Description/Methods: A 61 year-old male with a past medical history of coronary artery disease, aortic stenosis, type 2 diabetes mellitus and chronic hepatitis with cirrhosis (of unknown etiology from prior workup) presented with lethargy and fevers. Physical exam was significant for jaundice and tender hepatosplenomegaly. Bloodwork showed: lymphopenia, neutropenia, direct hyperbilirubinemia, and transaminitis. Right upper quadrant ultrasound showed a diffusely echogenic nodular liver with gallbladder dilation in the absence of biliary ductal dilation. Patient was empirically treated for ascending cholangitis with broad spectrum antibiotics and was later found to have E.coli bacteremia. Given patients septic state he underwent percutaneous cholecystostomy tube placement without complication. A complete infectious, hematologic, and hepatologic workup found negative/normal: CMV, EBV, TB, HIV, COVID-19, parvovirus B19, viral hepatitis, DAT, ANA, RF, ANCA, AMA, ASMA, ceruloplasmin, alpha-1 antitrypsin, and C282Y/S/6/5/C. Fibrinogen, ferritin, D-dimer, triglycerides, CXCL-9, and CD-25 were found to be abnormal. Patient's former records were reviewed and showed: cirrhosis with lymphocytic infiltrate on liver biopsy, a normal endoscopy, and an inconclusive bone marrow biopsy only showing clusters of histiocytes. The patient met criteria for HLH and was started on dexamethasone and etoposide. The patients condition continued to worsen and he was transferred to a tertiary care center where he was diagnosed with T-cell histiocyterich diffuse large B-cell lymphoma on repeated biopsy with HLH. Discussion: In patients with high-grade fevers of unknown etiology and multi-organ failure, HLH should be on the differential despite the rarity of adult cases. This case represents an unconventional presentation of HLH in the setting of a confounding bacteremia from ascending cholangitis and underlying B-cell Lymphoma.