Sohrab Zand, MBBCh, Sristee Niraula, MD Cayuga Medical Center, Ithaca, NY
Introduction: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening condition characterized by the excessive activation of macrophages and the absence of regulatory factors, such as Natural Killer cells, which normally serve to downregulate immune responses. Its association with alcoholic hepatitis (AH) is not well documented.
Case Description/Methods: A 26-year-old female with a recent hospital admission due to AH presented with worsening dyspnea and right upper quadrant pain since her previous discharge four days ago. The patient was hypoxic, requiring 6 liters of oxygen, jaundiced, and had abdominal distension. Initial investigations revealed leukocytosis with neutrophilic prominence, macrocytic anemia, thrombocytopenia, an elevated INR of 2.57, a direct bilirubin level of 7.5 mg/dL, an indirect bilirubin level of 12.0 mg/dL, an AST of 100 U/L, an ALT of 39 U/L, normal alkaline phosphatase, an albumin level of 3.0 g/dL, normal serum alcohol levels, and a negative PEth test. The chest computed tomography (CT) angiography scan showed extensive ground-glass opacification and interlobular septal thickening in both lungs, and the abdominal CT scan showed moderate ascites and splenomegaly. The patient’s hospital course was complicated by acute renal failure requiring dialysis, and acute respiratory distress syndrome, necessitating the need for intubation. Further workup revealed a ferritin level of 484.6 ng/mL, a fibrinogen level of 119.1 mg/dL, an IL-2 receptor soluble count of 8482.1 pg/mL, and a CXCL9 count of 4247 pg/mL. Given her poor prognosis, the patient was transitioned to comfort care and eventually succumbed to their condition. Histologic samples taken during the autopsy were consistent with acute liver failure, acute pancreatitis, acute tubular necrosis of the kidney, and diffuse alveolar damage.
Discussion: This case supports the necessity of early consideration of HLH in patients with severe AH with multiorgan involvement and worsening cytopenia. Our patient met five out of the nine criteria for HLH, meeting the diagnostic requirement. The multi-organ involvement seen in the autopsy report may have been attributed to a significant cytokine storm resulting from HLH. The involvement of macrophages in innate immunity along with the dysregulation of innate immunity seen in patients with AH raises the possibility the AH may act as a catalyst for HLH. Further cases are required to explore the association between severe AH and HLH.
Figure: The chest CT scan of the patient reveals extensive ground-glass opacification and thickening of the interlobular septa in both lungs
Disclosures:
Sohrab Zand indicated no relevant financial relationships.
Sristee Niraula indicated no relevant financial relationships.
Sohrab Zand, MBBCh, Sristee Niraula, MD. P4857 - A Fatal Case of Alcoholic Hepatitis Complicated by Hemophagocytic Lymphohistiocytosis, ACG 2024 Annual Scientific Meeting Abstracts. Philadelphia, PA: American College of Gastroenterology.
