Hepatic Langerhans cell histiocytosis: A review

Abstract

Hepatic Langerhans cell histiocytosis (LCH) is characterized by proliferation and accumulation of Langerhans cells in the liver, causing liver dysfunction or forming a mass lesion. The liver can be involved in isolation, or be affected along with other organs. A common clinical hepatic presentation is cholestasis with pruritis, fatigue and direct hyperbilirubinemia. In late stages, there may be hypoalbuminemia. Liver biopsy may be required for the diagnosis of hepatic LCH. Histologic finding may be diverse, including lobular Langerhans cell infiltrate with mixed inflammatory background, primary biliary cholangitis-like pattern, sclerosing cholangitis-like pattern, and even cirrhosis at later stages. Because of its non-specific injury patterns with broad differential diagnosis, establishing a diagnosis of hepatic LCH can be challenging. Hepatic LCH can easily be missed unless this diagnosis is considered at the time of biopsy interpretation. A definitive diagnosis relies on positive staining with CD1a and S100 antigen. Liver involvement is a high risk feature in LCH. The overall prognosis of hepatic LCH is poor. Treating at an early stage may improve the outcome. Systemic chemotherapy is the mainstay of treatment and liver transplantation may be offered. New molecular markers involved in pathogenesis of LCH are being explored with a potential for targeted therapy. However, further studies are needed to improve outcome.

Keywords: CD1a; Cholangitis; Langerhans cell; Liver.

Figure 1

Hepatic Langerhans cell histiocytosis mimicking primary biliary cholangitis. A: Portal non-necrotizing granulomatous inflammation encasing a duct (Hematoxylin and eosin, 200 ×); B: CD1a immunostain highlights Langerhans cells surrounding the duct (CD1a, 200 ×). Image A was originally published in Cureus. Citation: Li H, Ells P, Arslan ME, Robstad KA, Lee H. Hepatic Langerhans Cell Histiocytosis (LCH) Presenting as a Harbinger of Multisystem LCH. Cureus 2020; 12: e8591 [PMID: 32676232 DOI: 10.7759/cureus.8591]. Copyright © The Authors 2020. Published by Cureus, Inc.[21].