title: Acute Sickle Hepatic Crisis after Liver Transplantation in a Patient with Hb SC Disease.
abstract: Acute sickle hepatic crisis (ASHC) has been observed in approximately 10% of patients with sickle cell disease. It occurs predominantly in patients with homozygous (Hb SS) sickle cell anemia and to a lesser degree in patients with Hb SC disease, sickle cell trait, and Hb S beta thalassemia. Patients commonly present with jaundice, right upper quadrant pain, nausea, low-grade fever, tender hepatomegaly, and mild to moderate elevations in serum AST, ALT, and bilirubin. We describe the case of a patient with a history of hemoglobin SC disease and cirrhosis caused by hepatitis C presenting approximately 1 year after liver transplantation with an ASHC. The diagnosis was confirmed by liver biopsy. Our patient was treated with RBC exchange transfusions, IV hydration, and analgesia and made a complete recovery. Only a limited number of patients with sickle cell disease have received liver transplants, and, to our knowledge, this is the first case of ASHC after transplantation in a patient with Hb SC disease.
authors: Department of Internal Medicine, The University of Tennessee Health Science Center, Memphis, TN 38104, USA.
authors: J H
authors: JH
authors: Gillis
authors: Department of Surgery, The University of Tennessee Health Science Center, Memphis, TN 38104, USA ; Methodist University Hospital Transplant Institute, Memphis, TN 38104, USA.
authors: S K
authors: SK
authors: Satapathy
authors: Department of Internal Medicine, The University of Tennessee Health Science Center, Memphis, TN 38104, USA.
authors: L
authors: L
authors: Parsa
authors: Department of Surgery, The University of Tennessee Health Science Center, Memphis, TN 38104, USA ; Methodist University Hospital Transplant Institute, Memphis, TN 38104, USA.
authors: P B
authors: PB
authors: Sylvestre
authors: Department of Surgery, The University of Tennessee Health Science Center, Memphis, TN 38104, USA ; Methodist University Hospital Transplant Institute, Memphis, TN 38104, USA.
authors: N
authors: N
authors: Dbouk