Fatal vanishing bile duct syndrome after cord blood transplantation mimicking HBV reactivation.

BACKGROUND

Vanishing bile duct syndrome (VBDS) is a rare but life-threatening cause of cholestatic liver dysfunction characterized by progressive bile duct loss. While associated with various triggers, its occurrence after hematopoietic stem cell transplantation is uncommon and diagnostically challenging, especially when mimicking hepatitis B virus (HBV) reactivation.

CASE PRESENTATION: We report a 71-year-old woman who developed VBDS following umbilical cord blood transplantation for myelodysplastic syndrome. Five years post-transplant, she presented with jaundice and elevated HBV-DNA levels, initially raising suspicion for HBV reactivation. Despite antiviral therapy, her liver function deteriorated. Imaging revealed widespread lymphadenopathy, and biopsies confirmed classical Hodgkin lymphoma-type post-transplant lymphoproliferative disorder (PTLD). Liver biopsy demonstrated marked bile duct loss, consistent with VBDS. Despite initiation of chemotherapy, hepatic dysfunction necessitated dose reduction, and the patient succumbed 93 days after treatment initiation.

CONCLUSION

This is the first reported case of VBDS associated with PTLD. The clinical course underscores the importance of distinguishing VBDS from HBV reactivation in transplant recipients presenting with cholestatic liver dysfunction. Liver biopsy remains essential for accurate diagnosis. Early recognition is crucial, as progressive liver injury may compromise treatment and worsen outcomes. This case expands the differential diagnosis of post-transplant liver failure and highlights the potential for PTLD to trigger VBDS.