2350 An Unlikely Presentation of Diffuse Large B-Cell Lymphoma Manifesting as Hepatic Failure

Abstract

INTRODUCTION: Primary Hepatic Lymphoma (PHL) is a very rare subtype of Diffuse Large B-Cell Lymphoma (DLBCL), accounting for less than 1% of all cases. In rare instances, a patient can present with fulminant hepatic failure. The prognosis is grim with an average survival of 10.7 days from admission. The mainstay of diagnosis is liver biopsy, which must be performed as early as possible to prevent progression to acute liver failure. CASE DESCRIPTION/METHODS: A 55-year-old male with no pertinent medical history presented with a two week history of abdominal pain, fever, lethargy and mild jaundice. He was a former smoker and drinker who denied any alcohol use for the past 15 years. Upon presentation, the patient’s labs showed acute liver injury, anemia, thrombocytopenia and acute kidney injury. The patient’s model for end-stage liver disease (MELD) was 29. He was subsequently admitted to the medical intensive care unit (ICU) where emergent liver transplant evaluation was initiated. A full work up for the etiology of his liver failure was initially unrevealing so a liver biopsy was performed, which came back positive for DLBCL. The patient was then started on a chemotherapeutic regimen consisting of cyclophosphamide, vincristine, etoposide and high dose steroids. The patient’s condition further deteriorated during his ICU stay, leading to the need for mechanical ventilation, vasopressor therapy for circulatory shock, continuous renal replacement therapy for worsening renal failure and transfusion of blood products for worsening coagulopathy. Despite all of these aggressive measures, the patient’s clinical status only continued to worsen. The family decided to institute comfort measures and the patient died on ICU day 12. DISCUSSION: DLBCL predominantly affects Caucasian men over the age of 50. Some viruses, specifically EBV, HBV and HCV, have shown correlation with Non-Hodgkin Lymphoma (NHL); our patient had serologies indicative of past exposure to EBV and HBV. The only way to definitively diagnose DLBCL is via liver biopsy with immunohistochemical staining that reveals CD20, CD45 and BCL-6. The prevailing treatment modality is the use of Dose Adjusted Etoposide, Prednisone, Oncovin, Cyclophosphamide, Hydorxydauorubicin and Rituximab (DA-EPOCH R) Therapy. Due to its poor prognosis, early diagnosis and initiation of treatment for PHL is crucial. For this reason, it must be considered early in the differential when evaluating a patient with acute liver failure.