A Rare Lymphoma Associated With a Ubiquitous Disease: A Case of Gastrointestinal Extra-Nodal Natural Killer/T-Cell Lymphoma

Document Type

Conference Proceeding

Publication Date

10-1-2024

Publication Title

Am J Gastroenterol

Abstract

Introduction: Extra-nodal Natural Killer/T-cell Lymphoma (ENKTL) is a rare extra-nodal lymphoma commonly associated with Epstein-Barr Virus (EBV) infection. This malignancy usually presents in the facial and nasopharyngeal areas. We present an unusual case of ENKTL with a primary locus in the liver. Case Description/Methods: A 38-year-old woman with a medical history of type 1 diabetes mellitus, congestive heart failure, and necrotizing pneumonia is admitted for abnormal liver function tests (LFTs) and accompanying severe abdominal pain. The patient's LFTs show alanine aminotransferase (ALT) 99, aspartate aminotransferase (AST) 179, alkaline phosphatase (ALP) 541, and total bilirubin 0.4. Abdominal computed tomography (CT) shows an ill-defined hyperintensity in the left hepatic lobe of the liver and moderate ascites. Paracentesis is significant for 45% abnormal lymphoid cells possessing NK phenotype. No clonal T-cell receptor gene rearrangement is seen in the sample. Lab evaluation by in-situ hybridization reveals EBV viremia with an elevated viral load of 1,070,006 international units/milliliter. The patient's coagulopathy and liver function continue to worsen. Liver biopsy specimens are obtained and confirm extra-nodal NK-cell lymphoma causing acute liver failure and shock. Chemotherapy is initiated with methotrexate, ifosfamide, and etoposide (mSMILE). The patient soon develops disseminated intravascular coagulation. Tachypnea and hypoxia develop with bilateral pleural effusions and diffuse volume overload requiring thoracentesis. While undergoing chemotherapy, the patient develops a neutropenic fever thought likely due to GI translocation, and requires frequent transfusions due to hematochezia. Due to her precipitous decline, she is transitioned to hospice care less than one month after her initial presentation. Discussion: ENKTL is a malignancy most frequently associated with EBV infection. Though a few potential genetic predispositions have been identified, a direct causal link between ENKTL development and associated EBV infection has yet to be defined. Gastrointestinal presentation of ENKTL is uncommon, and its comparatively worse prognosis is not well understood.

Volume

119

Issue

10

First Page

S2924

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