P1156 - A Challenging Case of Hemophagocytic Lymphohistiocytosis (HLH) Presenting With Acute Liver Failure (ALF): Diagnostic and Therapeutic Dilemmas

Muhammad Taha Khan, MD¹, Bishr Swar, MD¹, Gina Moon, MD¹, Grace Braimoh, MD², Ruqaiyyah Hamid, MD³, Ravindranauth Sawh, MBBS, DM⁴, Bradley Gehrs, MD², Tommy E.. Yates, DO⁴, Himanshu Bhardwaj, MD^2
1Oklahoma University Health Sciences Center, Oklahoma City, OK; ²University of Oklahoma College of Medicine, Oklahoma City, OK; ³Piedmont Regional Medical Center, Newark, CA; ⁴Oklahoma City VA Healthcare System, Oklahoma City, OK

Introduction: HLH is a rare aggressive life-threatening syndrome of excessive immune activation which commonly impacts the liver. This case report explores the therapeutic and diagnostic dilemmas encountered in a critically ill patient with suspected HLH, underlying hepatic dysfunction and concurrent infections, ultimately impacting patient prognosis.

Case Description/Methods: A 46-year-old male with Acute Hepatitis of multifactorial etiology was admitted to the ICU with Septic Shock caused by ESBL E. coli Bacteremia and CMV Viremia, accompanied by deteriorating hepatic function and suspected HLH. An H score of 178 (54-70% probability of HLH) prompted consultation with Hematology. 4 of 8 HLH-2004 diagnostic criteria (Table 1) were met, pending Bone Marrow Biopsy. Initial management involved IV Ertapenem, Ganciclovir, and CVC pressor support. A recent Liver Biopsy (Fig. 1) indicated Acute Steatohepatitis, DILI, or Toxic Liver Injury, while MRCP excluded intrahepatic lesions or obstruction. Transplant Hepatology deemed the patient ineligible for transplantation due to concurrent infections, concern for HLH, and poor prognosis. Clinical deterioration led to Acute Liver Failure, prompting initiation of Dexamethasone for HLH. Bone marrow biopsy (Fig. 2) revealed infrequent Hematophagocytosis without excluding HLH. Tumor Board deferred Etoposide due to underlying liver failure and decreased suspicion of HLH based on bone marrow findings. Initially, the patient showed improvement but eventually succumbed to multiple organ failure after a 2-week hospital course.

Discussion: Early recognition and initiation of therapy are crucial for managing HLH. Underlying liver disease and concurrent infections can complicate diagnosis and treatment. However, a high level of suspicion, combined with supportive H score/HLH-2004 criteria in critically ill patients, justifies the initiation of therapy without awaiting advanced testing. Management is primarily comprised of Etoposide and Dexamethasone. In severe Liver Disease, use of Etoposide can be challenging due to its partial liver metabolism. Therefore, either reducing the Etoposide dose or alternatively, Alemtuzumab can be considered. Response to therapy can be followed by trending Serum Ferritin, as shown with our patient in Table 1. The role of Liver Transplantation in HLH presenting with Acute Liver Failure remains limited due to poor prognosis.




Disclosures:


Muhammad Taha Khan, MD¹, Bishr Swar, MD¹, Gina Moon, MD¹, Grace Braimoh, MD², Ruqaiyyah Hamid, MD³, Ravindranauth Sawh, MBBS, DM⁴, Bradley Gehrs, MD², Tommy E.. Yates, DO⁴, Himanshu Bhardwaj, MD². P1156 - A Challenging Case of Hemophagocytic Lymphohistiocytosis (HLH) Presenting With Acute Liver Failure (ALF): Diagnostic and Therapeutic Dilemmas, ACG 2023 Annual Scientific Meeting Abstracts. Vancouver, BC, Canada: American College of Gastroenterology.