Current perspectives on the role of liver transplantation for Langerhans cell histiocytosis: A narrative review

Abstract

Langerhans cell histiocytosis (LCH) is a malignant disease of the histiocytes involving various organ systems. The spectrum of liver involvement in LCH ranges from mild transaminitis to end-stage liver disease. The hallmark of hepatic LCH is secondary sclerosing cholangitis, which manifests due to a progressive destruction of the biliary tree by malignant histiocytes. Chemotherapy remains the mainstay of treatment for active LCH. Early recognition, diagnosis and a systematic approach to the management of LCH can ameliorate the disease process. Nonetheless, the liver involvement in these patients may progress despite the LCH being in remission. Liver transplantation (LT) remains central in the management of such patients. Various facets of the management of LCH, especially those with liver involvement remain unclear. Furthermore, aspects of LT in LCH with regards to the indication, timing and post-LT management, including immunosuppression and adjuvant therapy, remain undefined. This review summarises the current evidence and discusses the practical aspects of the role of LT in the management of LCH.

Keywords: Langerhans cell histiocytosis; Liver transplantation; Outcomes; Management; Chemotherapy; Ethics

Core Tip: Involvement of the liver in Langerhans cell histiocytosis (LCH) is considered a high-risk disease and the management algorithm needs to factor in the malignancy and the severity of liver disease. Liver transplantation (LT) is usually offered to LCH patients in remission with decompensated liver disease. However, given the paucity of currently available literature, the role of LT in LCH remains undefined. There is hence a need for large collaborative, multicentre studies to provide recommendations on the management algorithm for LCH.