2382 Successful Hepatitis C Virus Antibody-Positive Donor Liver Transplantation in Acute Sickle Cell Intrahepatic Cholestasis: A Case Report

Abstract

INTRODUCTION: Sickle cell hepatopathy (SCH) is the umbrella term referring to liver dysfunction in sickle cell disease (SCD) patients. We report a case of acute intrahepatic cholestasis (ASCIH) with underlying cirrhosis managed with hepatitis C virus (HCV) antibody-positive donor liver transplantation (LT). CASE DESCRIPTION/METHODS: A 29-year-old African American male with known medical history of SCD (Hb SS) and cirrhosis presented to the medical intensive liver unit with vaso-occlusive pain crises and jaundice. Patient was afebrile with benign exam. Labs were consistent with obstructive jaundice (total serum bilirubin of 57 mg/dL, direct serum bilirubin of 30 mg/dL), and elevated liver enzymes (alkaline phosphatase 306 U/L, aspartate transaminase 227 U/L and alanine transaminase 54 U/L). He had significant leukocytosis 38.6 k/uL, low hemoglobin 6.3 g/dL and thrombocytopenia 39 k/uL. Sodium level was 149 mg/dl and INR was 2.3. Computed tomography scan showed changes consistent with cirrhosis but no evidence of ductal dilatation. He developed encephalopathy after 24 hours of presentation, acute kidney injury (serum creatinine 3.57 mg/dl) and respiratory failure requiring endotracheal intubation. He was diagnosed with acute liver failure secondary to ASCIH after a non-revealing extensive workup for acute liver failure. Exchange transfusion was started without improvement in hepatic function or mental status. His MELD-Na (Model for End-Stage Liver Disease-Sodium) score was 43 and thus patient was listed for urgent LT. He received an HCV positive donor liver after 9 days of hospitalization. Explanted liver biopsy showed widespread sinusoidal dilatation with innumerable clusters of sickled red blood cells, scattered pigmented histiocytes, and cholestasis. Following transplant, exchange transfusion was resumed and immunosuppression treatment started with mycophenolate, tacrolimus and steroid taper. Glecaprevir/Pibrentasvir started for high HCV RNA titer. He was discharged after 23 days with total bilirubin of 4.2 mg/dL and normalization of his liver enzymes. He was doing well during his 4 months follow up. DISCUSSION: We present a rare case of acute sickle cell intrahepatic cholestasis treated with successful LT. This represents the 1st case of a positive HCV donor LT and the 20th LT case in SCD. ASCIH is the most severe, often fatal, form of SCH. Our goal with this case is to increase the awareness of this important condition and emphasize the role of liver transplantation as a potential treatment.