2009 Southern Regional Meeting Abstracts

Purpose of Study: Sickle cell crises manifest as multiorgan involvement secondary to an amino acid substitution of valine for glutamic acid resulting in polymerization of deoxy hemoglobin and subsequent vaso-occlusion. Common presentations include bone infarction, acute chest syndrome, and cerebrovascular events; however, hepatic involvement occurs in approximately 10 percent of sickle cell crises and results in liver sequelae. Acute hepatic crises with hepatic failure can pose a diagnostic challenge as well as indicate for quick therapeutic intervention. We report a case of hepatic sickle crisis where early intervention resulted in a favorable outcome.
Methods Used: 33-year-old African American male with a history of sickle cell disease and sarcoidosis presented with chest pain radiating to the epigastrium and shortness of breath. Physical exam revealed him tachycardic, afebrile, and oxygen saturations at 92% on room air. Additionally, there was jaundiced skin, lung crackles, and epigastric distention with right upper quadrant tenderness. The liver edge was palpable 10 cm below the costal margin and extended to midline. Laboratory studies were WBC 15, hemoglobin 7.9, platelets 330, total bilirubin 35.2, AST 207, ALT 118, alkaline phosphatase 664, total protein 7.35, albumin 2.5, and PT/INR 18.6/1.5. Chest radiography was suggestive of lobar pneumonia and antibiotics were begun. Right upper quadrant ultrasound revealed no evidence of biliary ductal dilatation; however, the liver measured 19 cm in diameter. Supportive treatment and antibiotics continued until hospital day 3 when there was worsening mental status, respiratory distress, coagulopathy, rise in bilirubin, and a hemoglobin decrease. At this time, acute hepatic sickle crisis with hepatic failure and sequestration was diagnosed. Prompt exchange transfusion was performed and rapid improvement followed with transaminases at baseline. Our patient was discharged home in stable condition.
Summary of Results: While sickle cell hepatopathy is infrequent, acute hepatic crises possess devastating sequelae. Although diagnostic criteria still remain undefined, early intervention with exchange transfusion can reduce mortality.
Conclusions: In reporting this case, we illustrate the importance of early diagnostic recognition and exchange transfusion to avoid further devastating outcomes.