Epistaxis: A Rare Presentation of Sickle Cell Intrahepatic Cholestasis

Sickle cell hepatopathy (SCH) is an umbrella term relating to liver disease in sickle cell disease (SCD). This term ranges from common etiologies such as cholelithiasis to disease-specific causes such as sickle cell intrahepatic cholestasis (SCIC), a rare but significant complication of SCD capable of progressing to liver failure and consideration of transplantation. We report the case of a 24-year-old male with SCD who presented with jaundice, encephalopathy, uncontrollable epistaxis, and pseudohematemesis and was found to have hyperbilirubinemia, coagulopathy, portal hypertension, and acute kidney injury (AKI). This presentation was concerning for SCIC. Initial management included transfusions and a trial of apheresis. Liver biopsy revealed sinusoidal red cell sickling, fibrosis, and ductopenia, consistent with findings of SCIC. Due to ongoing complications, recurrent admissions, and symptomatic coagulopathy, the patient underwent liver transplantation which was complicated by perihepatic hematoma and stroke, necessitating extensive rehabilitation. This case emphasizes the importance of early diagnostic workup and prompt, multidisciplinary management of SCIC to mitigate risks of liver failure and need for transplant.