P3980 - Hemophagocytic Lymphohistiocytosis in Inflammatory Bowel Disease: A Rare Entity

Simin Khan, MD¹, Kabir Husain, DO², Hassaan A. Zia, MD^2
1Louisiana State University Health, Shreveport, LA; ²LSU Health Sciences Center, Shreveport, LA

Introduction: Acute liver failure (ALF) is a dramatic and highly unpredictable clinical syndrome defined by the sudden onset of coagulopathy and encephalopathy. One of the leading causes of ALF is acetaminophen (APAP) overdose with a 66 % chance of recovery with early administration of N-acetylcysteine (NAC) and supportive care. Failure to improve should prompt a search for other causes. We present a case of Hemophagocytic lymphohistiocytosis (HLH) masquerading as ALF.

Case Description/Methods: A 53-year-old African American male with hypertension, chronic kidney disease (CKD) and Crohn's disease, presented to the emergency department with lethargy and poor appetite for 3 days. He was hypotensive to 70/40s, not responsive to fluids, and was started on norepinephrine. His initial labs were significant for ALT 5000 U/L, AST was too high to calculate, INR 4.95, ammonia 517 umol/L, total bilirubin 3.6 mg/dl, ALP 158 U/L, glucose 61 mg/dl, ferritin >33,511 ng/ml and LDH 306 U/L. Tylenol level was 36.9 µg/ml and the patient was started on NAC. His hospital course was further complicated by altered sensorium and upper GI bleeding. CT Head showed cerebral edema, however, his mental status gradually improved. EGD showed grade A esophagitis, hiatal hernia, and erosions with ulcers. Octreotide and NAC were discontinued on day 4. On day 15, he had an acute drop in platelets from 68K to 29K and a drop in Hb from 8 mg/dl to 6.5 mg/dl. Repeat EGD did not show any active bleeding. He continued to have bicytopenia with no other overt signs of bleeding. Platelet count dropped to 20K even after multiple transfusions. Hematology and Rheumatology were consulted. Interlukin-2 was 7902.2 pg/ml and Interleukin-18 was 1427 pg/ml. HLH was suspected given underlying IBD. Liver enzymes continued to remain elevated. On day 19, he was started on steroids and his liver enzymes showed significant improvement gradually as seen in Graph 1. Bone marrow biopsy was mildly hypocellular with plasmacytosis and kappa light chain excess. He had been on steroids for quite a while prior to the biopsy which was thought to be the cause for lack of evidence of hemophagocytosis.

Discussion: HLH is a rare condition that can be caused by a primary or acquired disorder of uncontrolled immune response. Liver injury is a common complication of HLH; however, HLH presenting as ALF has rarely been reported in adults. Despite treatment of APAP toxicity, failure in improvement of clinical and laboratory parameters should prompt search for an alternative etiology.



Disclosures:


Simin Khan, MD¹, Kabir Husain, DO², Hassaan A. Zia, MD². P3980 - Hemophagocytic Lymphohistiocytosis in Inflammatory Bowel Disease: A Rare Entity, ACG 2023 Annual Scientific Meeting Abstracts. Vancouver, BC, Canada: American College of Gastroenterology.