Successful outcome of acute sickle cell intrahepatic cholestasis in a patient with sickle/beta+ thalassaemia and chronic hepatitis C virus infection

Abstract

Acute sickle cell intrahepatic cholestasis (SCIC) is a rare but usually fatal complication of sickle cell disease (SCD), which has mostly been described in homozygous for hemoglobin S patients. It is characterized by acute hepatomegaly, striking jaundice, bleeding diathesis and increasing encephalopathy [ 1 Banerjee S Owen C Chopra S Sickle cell hepatopathy. Hepatology. 2001; 33: 1021-1028 Crossref PubMed Scopus (126) Google Scholar , 2 Khurshid I Anderson L Downie G.H Pape G.S Sickle cell disease, extreme hyperbilirubinemia, and pericardial tamponade: case report and review of the literature. Crit Care Med. 2002; 30: 2363-2367 Crossref PubMed Scopus (28) Google Scholar ]. On the other hand, hepatitis C virus (HCV) infection is common (up to 30.3%) among transfused patients with SCD [ 1 Banerjee S Owen C Chopra S Sickle cell hepatopathy. Hepatology. 2001; 33: 1021-1028 Crossref PubMed Scopus (126) Google Scholar , 3 Hasan M.F Marsh F Posner G Bellevue R Dosik H Suatengco R et al. Chronic hepatitis C in patients with sickle cell disease. Am J Gastroenterol. 1996; 91: 1204-1206 PubMed Google Scholar ]. However, the development of acute SCIC in an HCV infected patient with s/β+ thalassaemia has never been reported previously.