# Resolution of vanishing bile duct syndrome in a patient associated with refractory hodgkin lymphoma following Anti–PD-1 therapy: a case report and literature review

## TL;DR

A patient with a rare liver condition caused by Hodgkin lymphoma showed full recovery after treatment with an immune checkpoint inhibitor.

## Contribution

This is the first report of successful treatment of Hodgkin lymphoma-related vanishing bile duct syndrome using a PD-1 inhibitor.

## Key findings

- Pembrolizumab induced complete metabolic remission of refractory Hodgkin lymphoma.
- Liver function normalized without immune-related hepatic adverse events.
- Immune checkpoint inhibitors may be a viable treatment for HL-related VBDS.

## Abstract

Vanishing bile duct syndrome (VBDS) is a rare, and often fatal complication of Hodgkin lymphoma (HL), characterized by progressive intrahepatic bile duct loss and severe cholestasis. Management remains ill-defined, particularly in patients with refractory HL and significant hepatic dysfunction. We present a case of a young woman with biopsy-proven VBDS who experienced disease progression and worsening cholestasis despite second-line chemotherapy, corticosteroids, and brentuximab vedotin. Salvage therapy with pembrolizumab was initiated, resulting in a complete metabolic remission of HL and normalization of liver function. Notably, the patient did not experience immune-related hepatic adverse events. To our knowledge, this is the first report of HL-related VBDS successfully treated with programmed death-1 blockade. This case suggests that immune checkpoint inhibitors may be a viable therapeutic option for patients with HL-related VBDS, even in the setting of severe hepatic dysfunction.