Liver Transplantation for Langerhans Cell Histiocytosis: A U.S. Population-Based Analysis and Systematic Review of the Literature.

Introduction Langerhans cell histiocytosis (LCH) is the most common histiocytic disorder. Liver involvement is seen in 10.1%-19.8% of patients with LCH and can lead to secondary sclerosing cholangitis requiring liver transplantation (LT). We aimed to describe the characteristics and outcomes of patients undergoing LT for LCH. Methods All patients undergoing a first LT for LCH in the Unites States were identified in the Scientific Registry of Transplant Recipients (SRTR) database (1987-2018). The Kaplan Meier method and log-rank tests evaluated post-LT survival. A systematic literature review was performed according to the PRISMA guidelines. Results Sixty LCH LT recipients were identified in SRTR. Fifty-five patients (91.7%) were children, median total bilirubin at LT was 5.8 mg/dL (IQR: 2.7-12.9), and 49 (81.7%) underwent deceased donor LT (DDLT). The 1-, 3-, and 5-year patient survival rates were 86.6%, 82.4%, and 82.4%. The systematic review yielded 26 articles reporting on 50 patients. Forty-one were children (82%), 90% had multisystem LCH, and most patients underwent DDLT (91.9%, n=34/37). Pre-LT chemotherapy was administered in 74% and steroids in 71.7% (n=33/46) and recurrence of LCH to the liver was reported in 8%. Eleven of the 50 patients (22.0%) died over a median follow-up of 25.2 months (IQR: 9.0-51.6) and the 1-year patient survival rate was 79.4%. Conclusion LT can be considered as a feasible life-saving option for the management of liver failure secondary to LCH in well-selected patients.