# Liver Transplantation for Isolated Langerhans Cell Histiocytosis in an Adult

## TL;DR

A 56-year-old man with liver-only Langerhans cell histiocytosis underwent successful liver transplantation and remained stable for three years.

## Contribution

This case report highlights liver transplantation as a potential treatment for isolated adult-onset LCH involving the liver.

## Key findings

- The patient had unisystemic LCH limited to the liver, confirmed by pathological examination.
- Liver transplantation led to stable liver function without recurrence or additional therapy for three years.
- LCH was not detected in other systems, as confirmed by negative bone marrow and bone scan results.

## Abstract

Langerhans cell histiocytosis (LCH) is a rare disease of proliferation of histiocytic disorder composed of histologically bland Langerhans cells mixed with reactive mononuclear and granulocytic cells, and often accompanied by eosinophils. These cells are characterized by expression of CD1a, S-100 and Langerin proteins. The clinical presentation ranges from indolent to aggressive, depending on the anatomic site involved which can be unifocal, multifocal, unisystemic, or multifocal and multisystemic disease. Cases involving LCH disease and treatment involving the liver are rare, especially in adult patients. Herein, we discuss a case of a 56-year-old male patient who presented with jaundice, acute abdominal pain, and a history of elevated liver function tests assumed to be caused by fatty liver disease. However, a computed tomography (CT) scan revealed a cholangiocarcinoma with associated biliary dilatation and cirrhosis. Pathological examination revealed Langerhans cell involvement. Negative bone marrow biopsy and bone scan indicated that the patient was indeed suffering from unisystemic LCH with isolated liver involvement causing cirrhosis. Patient underwent orthotopic liver transplantation (LT) and has since shown stable liver function without external therapy for 3 years.