P4809 - Acute Liver Failure and Cytotoxicity Secondary to Natural Killer Cell Leukemia

Ahmed Fares, MD¹, Saleh Alghsoon, MBBS¹, Mostafa T.. Mohammed, MD, PhD¹, Daniel Rust, MD¹, Raffi Karagozian, MD^2
1Tufts Medical Center, Boston, MA; ²Tufts Medicine, Boston, MA

Introduction: Aggressive NK-cell leukemia (ANKL) is a rare and severe hematological malignancy characterized by the proliferation of natural killer (NK) cells, rapid clinical decline and poor prognosis. NK cells, essential in innate immunity, become pathogenic in ANKL and cause liver cytotoxicity and injury. The association with Epstein–Barr virus (EBV) further complicates the clinical presentation of ANKL, suggesting a viral role in the pathogenesis of ANKL. This report presents a case of acute liver failure with biopsy demonstrating ANKL and an underlying EBV infection.

Case Description/Methods: A 24-year-old female with a history of acute fatty liver of pregnancy (AFLP) presented with sepsis and possible recurrent AFLP during a second pregnancy. Initial laboratory tests revealed significantly elevated liver function tests (LFTs). Liver biopsy showed lobular granulomatous inflammation, hepatocellular cholestasis, and macrovesicular steatosis (figure A). EBV-positive NK cells were identified, raising suspicion for ANKL (figure B). Peripheral blood flow cytometry and bone marrow biopsy confirmed an expanded NK cell population with high EBV viral load, supporting the diagnosis of ANKL (figure C). Treatment included ganciclovir, bortezomib, and alemtuzumab. Despite initial treatment, the patient’s condition deteriorated, resulting in disseminated intravascular coagulation and septic shock, leading to a transition to comfort measures and subsequent death.

Discussion: The liver is an essential organ in the immune response and hosts a high number of NK cells, making it essential in immune surveillance and pathogen defense. In ANKL, dysregulated NK cells are directed against hepatocytes, leading to severe liver injury. The interaction between liver NK cells and the hepatic environment is crucial in understanding inflammatory diseases causing liver damage. This case highlights a rare disease and the significant role of NK cells in liver pathology and their potential as therapeutic targets in liver diseases involving hematologic malignancies.



Disclosures:


Ahmed Fares, MD¹, Saleh Alghsoon, MBBS¹, Mostafa T.. Mohammed, MD, PhD¹, Daniel Rust, MD¹, Raffi Karagozian, MD². P4809 - Acute Liver Failure and Cytotoxicity Secondary to Natural Killer Cell Leukemia, ACG 2024 Annual Scientific Meeting Abstracts. Philadelphia, PA: American College of Gastroenterology.