Reversal of liver failure in sickle cell vaso-occlusive crisis.

A severe but unusual complication of sickle cell vaso-occlusive crisis is acute liver failure related to intrahepatic cholestasis. The outcome is usually fatal in adults. A case of reversible acute liver failure in a patient with s/beta+ thalassemia is reported. The patient was admitted to the intensive care unit because of major organ failure related to vascular occlusion phenomena. After blood-plasma transfusion and supportive therapy for acute liver failure, complete recovery was noted. A liver biopsy performed at the stage of recovery was compatible with intrahepatic cholestasis and sickling. Even though patients with s/beta+ thalassemia usually manifest milder symptoms, they rarely develop major organ failure such as acute liver failure.