Successful liver transplantation for acute sickle cell intrahepatic cholestasis: A case report and review of the literature  

作　　者：Motasem Alkhayyat Mohannad Abou Saleh Mohammad Zmaili Vedha Sanghi Tavankit Singh Carol Rouphael C Roberto Simons-Linares Carlos Romero-Marrero William D Carey Christina C Lindenmeyer 

机构地区：[1]Department of Internal Medicine,Cleveland Clinic,Cleveland,OH 44195,United States [2]Digestive Disease&Surgery Institute,Department of Gastroenterology,Hepatology&Nutrition,Cleveland Clinic,Cleveland,OH 44195,United States

出　　处：《World Journal of Hepatology》2020年第3期108-115,共8页世界肝病学杂志（英文版）（电子版）

摘　　要：BACKGROUND 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.CASE SUMMARY 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,nontender 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.CONCLUSION This case highlights the utility of LT as a viable treatment option for acute sickle cell intrahepatic cholestasis.BACKGROUND 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 23 rd 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.CASE SUMMARY 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, nontender 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.CONCLUSION This case highlights the utility of LT as a viable treatment option for acute sickle cell intrahepatic cholestasis.

关 键 词：Case report SICKLE cell HEPATOPATHY ACUTE INTRAHEPATIC CHOLESTASIS Liver transplant Hepatitis C virus POST-OPERATIVE surveillance 

分 类 号：R657.3[医药卫生—外科学]