Acute Sickle Cell Intrahepatic Cholestasis (SCIC) Successfully Treated with Exchange Blood Transfusion

Abstract

Sickle cell disease (SCD) is a common blood disorder known to have hepatobiliary complications. Sickle cell intrahepatic cholestasis (SCIC) is a rare, but severe manifestation that is often fatal if not recognized and treated appropriately. We present a case of acute SCIC successfully treated with exchange blood transfusion (EBT). A 40 year-old African-American woman with SCD presented with two days of myalgias, right upper-quadrant abdominal pain, nausea, and vomiting. She was afebrile, tachycardic, but saturating well on room air. She was alert and oriented with tender hepatomegaly and an otherwise unremarkable exam. Labs revealed leukocytosis, hemolytic anemia with numerous sickle cells on a peripheral smear, acute kidney injury, severe lactic acidosis, and hypoglycemia. She had an albumin of 3.5 g/dL, AST 2901 U/L, ALT 1116 U/L, ALP 160 U/L, Tbili 20.6 mg/dL, Dbili 15 mg/dL, and INR 2.8. Ultrasound of the liver revealed patent vasculature without cholelithiasis or common bile duct dilation. Viral, autoimmune, and toxin work-up were negative. She was diagnosed with acute liver injury due to SCIC. An EBT with 6 units of packed red blood cells was initiated on hospital day 2 and her hemoglobin S remained < 30%. She improved with supportive care and was discharged on hospital day 14. SCIC was first described in 1953 with few cases reported to date, roughly 50% of which were fatal. It is due to widespread sickling within hepatic sinusoids leading to ischemic and hypoxic injury. This results in ballooning of hepatocytes with resultant intracanalicular cholestasis and bile plugs. It presents with tender hepatomegaly, fever, leukocytosis, nausea, vomiting and can progress to acute liver failure. A direct hyperbilirubinemia is the characteristic finding that is seen with high levels of AST and ALT, reported to be as high as 3070 U/L and 6,680 U/L respectively. Early reports of SCIC were often fatal due to fulminant liver failure and bleeding. Anecdotal evidence has shown that EBT significantly improves outcomes with case reports of SCIC reversal if given early in the disease course. It has been proposed to maintain HbS levels < 30% which was achieved in our patient. SCIC is a rare complication of a common disease with a high mortality. Gastroenterologists should be aware because early recognition and initiation of exchange blood transfusion can improve mortality.