Menon J, Rammohan A, Vij M, Shanmugam N, Rela M. Current perspectives on the role of liver transplantation for Langerhans cell histiocytosis: A narrative review. World J Gastroenterol 2022; 28(30): 4044-4052 [PMID: 36157108 DOI: 10.3748/wjg.v28.i30.4044]
Corresponding Author of This Article
Ashwin Rammohan, FACS, FRCS, MCh, Attending Doctor, Institute of Liver Disease & Transplantation, Dr. Rela Institute & Medical Centre, Bharath Institute of Higher Education & Research, CLC Works Road, Chromepet, Chennai 600044, India. ashwinrammohan@gmail.com
Research Domain of This Article
Transplantation
Article-Type of This Article
Minireviews
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Jagadeesh Menon, Ashwin Rammohan, Mukul Vij, Naresh Shanmugam, Mohamed Rela, Institute of Liver Disease & Transplantation, Dr. Rela Institute & Medical Centre, Bharath Institute of Higher Education & Research, Chennai 600044, India
Author contributions: Menon J, Rammohan A, and Shanmugam N contributed to conception and design; Rammohan A, Menon J, and Rela M contributed to the acquisition, analysis, and interpretation of data; Menon J, Rammohan A, Vij M, and Shanmugam N drafted the article, and revised it critically for important intellectual content; Rela M gave the final approval of the version to be published.
Conflict-of-interest statement: There are no conflicts of interest to report.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Ashwin Rammohan, FACS, FRCS, MCh, Attending Doctor, Institute of Liver Disease & Transplantation, Dr. Rela Institute & Medical Centre, Bharath Institute of Higher Education & Research, CLC Works Road, Chromepet, Chennai 600044, India. ashwinrammohan@gmail.com
Received: January 9, 2022 Peer-review started: January 9, 2022 First decision: March 8, 2022 Revised: March 30, 2022 Accepted: July 19, 2022 Article in press: July 19, 2022 Published online: August 14, 2022
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.
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.
