329: A PERFECT STORM OF SICKLE CELL HEPATOPATHY

Abstract

Introduction: Intrahepatic cholestasis (SCIC) an uncommon but serious complication of vaso-occlusive crisis in sickle cell disease (SCD). Presenting with abdominal pain, hyperbilirubinemia, and transaminitis, and in severe cases with renal failure, encephalopathy, and coagulopathy. Early diagnosis is challenging but essential to improving outcomes. Herein, we report a case of a young patient with SCD who presented with SCIC requiring exchange transfusion which resulted in an eventual favorable outcome. Description: 18-year-old man with history of SCD presented for generalized abdominal pain. He complained of severe diffuse body pain. On admission, he had a total bilirubin (T.Bili) of 5.5 mg/dL, hemoglobin of 9.1 g/dL (baseline 8-9) and a clear chest x-ray. He was started on morphine PCA due to increasing pain and transfused one unit of packed red cells (pRBC). He subsequently developed a fever and had a chest x-ray concerning for acute chest syndrome. On day 2, hemoglobin dropped to 5.1 g/dL and he was transfused a further 2 units of pRBC. Over the course of day 2, he developed worsening kidney function and metabolic acidosis, so he was transferred to the medical intensive care unit. Overnight, he became increasingly encephalopathic and developed acute liver failure with AST 2317 IU/L, ALT of 1976 IU/L, INR of 4.7 and T.Bili of 13.3 mg/dL. An exchange transfusion was urgently arranged and initiated by morning of Day 3. His hospital course was complicated by ongoing encephalopathy, respiratory failure requiring mechanical ventilation and renal failure requiring hemodialysis. His total bilirubin, direct bilirubin and alkaline phosphatase peaked at 32.0 mg/dL, 30.0 mg/dL, 1024 IU/L, respectively. With further supportive care, he had recovery of liver function, was extubated, weaned off dialysis, and eventually discharged home. Discussion: This case illustrates the rapid and life threatening nature of SCIC and the importance of prompt initiation of exchange transfusion therapy. While SCIC is a challenging given its non-specific constellation of symptoms with significant overlap with several other hepatic processes, nevertheless it should be high on the differential in patients with SCD who develop hepatic abnormalities during vaso-occulsive crisis.