Sickle cell disease, extreme hyperbilirubinemia, and pericardial tamponade: case report and review of the literature.

Objective: Sickle cell disease is a relatively common disease seen predominantly in the African-American population with numerous important sequelae that require critical care management. We report a patient who presented with intrahepatic cholestasis, a rare and potentially lethal complication of sickle cell disease.

Design: Individual case report and review of the literature.

Setting: Medical intensive care unit of a tertiary care hospital.

Patient: A 37-yr-old African-American male, with known sickle cell disease, who developed fulminant hepatic failure with subsequent extreme hyperbilirubinemia, coagulopathy, and pericardial tamponade. Additional organ dysfunction included renal insufficiency, respiratory failure, and cardiac dysrhythmias.

Interventions: The patient underwent serial measurements of complete blood count, hepatic profile, coagulation profiles, and hemoglobin electrophoresis. The patient received exchange transfusion, and his hemoglobin S concentration gradually decreased. Coagulopathy and anemia were corrected with transfusion of fresh frozen plasma and packed red blood cells. Serum bilirubin and other hepatic variables gradually improved. Pericardial tamponade was suggested by right heart catheterization measurements and diagnosed by echocardiographic findings. The tamponade resolved after pericardiocentesis and was managed by drainage through the pericardial catheter. Pulmonary, renal, and cardiac sequelae resolved with intensive supportive care that included intubation, mechanical ventilation, pulmonary artery catheterization, continuous renal replacement therapy, and permanent cardiac pacemaker. The patient was discharged home on hospital day 23 and has not required further hospitalization.

Conclusion: Sickle cell intrahepatic cholestasis, a potentially fatal complication of sickle cell disease, can present with abdominal pain, acute hepatomegaly, coagulopathy, hyperbilirubinemia, and fulminant hepatic failure. Prompt recognition and early intervention with exchange transfusion and intensive supportive care of multiple organ dysfunction can result in a favorable outcome.