P2473 - Sickle Cell Crisis-Induced Intrahepatic Cholestasis: A Rare Yet Lethal Complication in Chronic Liver Disease Patients

Gagan P. Singh, MD¹, Suryanarayana Reddy Challa, MD¹, Kristen Miller, MD¹, Farshad Aduli, MD¹, Angesom Kibreab, MD^2
1Howard University Hospital, Washington, DC; ²Howard University Hospital, Washington, WA

Introduction: Sickle cell disease (SCD) can lead to liver complications such as acute hepatic crisis, acute and chronic Intrahepatic cholestasis (IHC). IHC is extremely rare, as reported only in case studies, is the most severe variant of SCD hepatic manifestation secondary to vaso-occlusion in the sinusoids leading to chronic hepatic ischemia and failure. Over time, these complications can induce chronic liver disease, necessitating liver transplantation. Despite more than 25% of SCD patients having chronic liver disease, liver transplants are infrequently performed, often leading to fatal outcomes without intervention.

Case Description/Methods: A 60-year-old male with a history of SCD, cholecystectomy, cirrhosis due to Alcohol use and untreated Hepatitis C presented with confusion and incoherent speech. Head CT was negative but was intubated for airway protection, and his hospital course was complicated by septic shock, DIC, hypoxic respiratory failure and renal failure. Laboratory work up showed increased total Bilirubin (60.5 mg/dl), aspartate aminotransferase (27IU/ml), and alkaline phosphatase (156 IU/ml), low haptoglobin, high LDH, and the presence of schistocytes. US abdomen showed nodular liver without intra or extra hepatic ductal dilation, no evidence of stones, mass, or portal vein thrombosis. Patient received IVIG and multiple blood transfusions without any improvement. Unfortunately, due to his hemodynamic instability we were unable to do exchange transfusions. Ultimately, the patient suffered a cardiac arrest and passed away.

Discussion: This case highlights the mortality risk in SCD patients with Intra Hepatic Cholestasis, especially when presenting with SCD crisis. Interventions like exchange transfusions and liver transplantation during the acuity of condition is very challenging. Understanding its pathophysiology, alongside early diagnosis, and effective medical management is crucial for improving outcomes. Based on our case we emphasize the importance of proactive liver transplantation evaluation in patents with SCD induced IHC to significantly reduce unexpected outcomes & mortality.



Disclosures:


Gagan P. Singh, MD¹, Suryanarayana Reddy Challa, MD¹, Kristen Miller, MD¹, Farshad Aduli, MD¹, Angesom Kibreab, MD². P2473 - Sickle Cell Crisis-Induced Intrahepatic Cholestasis: A Rare Yet Lethal Complication in Chronic Liver Disease Patients, ACG 2023 Annual Scientific Meeting Abstracts. Vancouver, BC, Canada: American College of Gastroenterology.