Acute Sickle Hepatic Crisis after Liver Transplantation in a Patient with Hb SC Disease

Case Reports in Transplantation
J H GillisN Dbouk

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.

References

May 9, 2001·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·S BanerjeeS Chopra
Feb 27, 2009·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·Don C RockeyUNKNOWN American Association for the Study of Liver Diseases
Aug 20, 2010·Transplantation·Guilherme F PeriniNelson Hamerschlak
Feb 26, 2014·Blood·Kate GardnerSwee Lay Thein

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Methods Mentioned

BETA
biopsy
electrophoresis
biopsies

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