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

Abstract EN

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.