Sickle cell intrahepatic cholestasis: approach to a difficult problem

Area of Science:

• Hematology
• Hepatology
• Critical Care Medicine

Background:

• Sickle cell intrahepatic cholestasis (SCIC) is a rare, life-threatening complication of sickle cell disease (SCD).
• SCIC presents with hepatomegaly, severe hyperbilirubinemia, coagulopathy, and acute liver failure.
• The underlying pathophysiology of SCIC remains unclear, with a historically poor prognosis.

Observation:

• This report details two cases of hemoglobin SS patients experiencing SCIC.
• Clinical features observed included severe jaundice, liver enlargement, and impaired blood clotting.
• Both patients presented with signs of acute liver dysfunction.

Findings:

• Exchange transfusion was identified as a critical therapeutic intervention.
• Supportive care focused on correcting coagulopathy and stabilizing liver function.
• Avoidance of surgical procedures was deemed essential for patient recovery.

Implications:

• Successful management of SCIC hinges on prompt exchange transfusion and comprehensive supportive care.
• Aggressive medical management, including avoidance of surgery, can lead to favorable outcomes in SCIC.
• These findings offer a potential treatment strategy for this rare but severe SCD complication.