Published online Mar 27, 2020. doi: 10.4254/wjh.v12.i3.108
Peer-review started: September 13, 2019
First decision: September 26, 2019
Revised: December 28, 2019
Accepted: February 17, 2020
Article in press: February 17, 2020
Published online: March 27, 2020
Processing time: 192 Days and 12.7 Hours
Sickle cell hepatopathy (SCH) is an inclusive term referring to any liver dysfunction among patients with sickle cell disease. Acute sickle cell intrahepatic cholestasis is one of the rarest and most fatal presentations of SCH. We present the 23rd reported case of liver transplantation (LT) for SCH; a rare case of acute sickle cell intrahepatic cholestasis managed with LT from a hepatitis C virus (HCV) nucleic acid amplification test positive donor.
A 29-year-old male with a past medical history of sickle cell disease presented with vaso-occlusive pain crisis. On examination, he had jaundice and a soft, non-tender abdomen. Initially he was alert and fully oriented; within 24 h he developed new-onset confusion. Laboratory evaluation was notable for hyperbilirubinemia, leukocytosis, anemia, thrombocytopenia, acute kidney injury and elevated international normalized ratio (INR). Imaging by ultrasound and computed tomography scan suggested a cirrhotic liver morphology with no evidence of biliary ductal dilatation. The patient was diagnosed with acute sickle cell intrahepatic cholestasis after excluding competing etiologies of acute liver injury. He underwent LT from an HCV nucleic acid amplification test positive donor 9 d after initial presentation. The liver explant was notable for widespread sinusoidal dilatation with innumerable clusters of sickled red blood cells and cholestasis. On postoperative day 3, HCV RNA was detectable in the patient's peripheral blood and anti-HCV therapy with glecaprevir/pibrentasvir was initiated on postoperative day 23. He subsequently achieved sustained virologic response after completing 3 mo of therapy and has been followed clinically for 12 mo post-transplant.
This case highlights the utility of LT as a viable treatment option for acute sickle cell intrahepatic cholestasis.
Core tip: Acute sickle cell intrahepatic cholestasis is a rare and life-threatening form of sickle cell hepatopathy, with a mortality rate approaching 40%. Patients typically present with fever, abdominal pain and jaundice. Rarely, it may progress to an acute liver failure phenotype, commonly associated with multi-system organ failure. Diagnosis is made after excluding other causes of acute liver injury. Treatment options include exchange blood transfusion and liver transplantation.