Plasmapheresis can improve clinical outcomes in patients with therapy resistant benign recurrent intrahepatic cholestasis: A case report.

BACKGROUND

Due to the rarity of benign recurrent intrahepatic cholestasis (BRIC), limited literature exists regarding the therapeutic benefit of plasmapheresis, particularly in the context of severe hyperbilirubinemia.

CASE REPORT: A 25-year-old female presented with jaundice and severe pruritus. Laboratory findings revealed a total bilirubin of 37 mg/dL and a direct bilirubin of 27 mg/dL. Alanine aminotransferase (ALT) was 31 U/L, aspartate aminotransferase (AST) was 35 U/L, and alkaline phosphatase was 175 U/L. The patient had a five-year history of recurrent episodes, previously resolving spontaneously or with medical treatment. Following liver biopsy diagnosis and failure of conventional therapy, she underwent plasmapheresis on alternate days. Four sessions of one plasma volume exchange, using albumin, were performed concurrently with medical treatment. The patient demonstrated rapid and significant improvement in pruritus and urine color, with no adverse events or need for fresh frozen plasma. Total bilirubin decreased progressively to 5 mg/dL, and direct bilirubin to 4 mg/dL.

CONCLUSION

Plasmapheresis appears to be a beneficial adjunct therapy for refractory BRIC, effectively reducing bilirubin levels and alleviating pruritus. This intervention may improve patient quality of life and potentially prevent renal complications associated with hyperbilirubinemia, serving as a bridge to liver transplantation, if necessary.