Liver Transplantation for Langerhans Cell Histiocytosis: A US Population‐Based Analysis and Systematic Review of the Literature. Issue 8 (14th July 2021)

• Record Type: Journal Article
• Authors: Ziogas, Ioannis A.; Kakos, Christos D.; Wu, W. Kelly; Montenovo, Martin I.; Matsuoka, Lea K.; Zarnegar‐Lumley, Sara; Alexopoulos, Sophoclis P.
• Is Part Of: Liver transplantation. Volume 27:Issue 8(2021)
• Page Start: 1181
• Page End: 1190
• Publication Date: 2021-07-14
• Subjects: Liver -- Transplantation -- Periodicals; Liver -- Diseases -- Periodicals; Liver Transplantation -- Periodicals; Foie -- Greffe -- Périodiques; 617.5560592
• DOI: 10.1002/lt.25995
• Languages: English
• ISSNs: 1527-6465

Abstract

Abstract : Langerhans cell histiocytosis (LCH) is the most common histiocytic disorder. Liver involvement is seen in 10.1% to 19.8% of patients with LCH and can lead to secondary sclerosing cholangitis requiring liver transplantation (LT). We describe the characteristics and outcomes of patients undergoing LT for LCH. All patients undergoing a first LT for LCH in the United States were identified in the Scientific Registry of Transplant Recipients (SRTR) database (1987‐2018). The Kaplan‐Meier curve method and log‐rank tests evaluated post‐LT survival. A systematic literature review was performed according to the Preferred Reporting Items for Systematic Reviews and Meta‐analysis (PRISMA) statement. A total of 60 LCH LT recipients were identified in the SRTR, and 55 patients (91.7%) were children with median total bilirubin levels at LT of 5.8 mg/dL (interquartile range [IQR], 2.7‐12.9). A total of 49 patients (81.7%) underwent deceased donor LT (DDLT). The 1‐year, 3‐year, and 5‐year patient survival rates were 86.6%, 82.4%, and 82.4%, respectively. The systematic review yielded 26 articles reporting on 50 patients. Of the patients, 41 were children (82.0%), 90.0% had multisystem LCH, and most patients underwent DDLT (91.9%; n = 34/37). Pre‐LT chemotherapy was administered in 74.0% and steroids in 71.7% (n = 33/46) of the patients, and a recurrence of LCH to the liver was reported in 8.0% of the patients. Of the 50 patients, 11 (22.0%) died during a median follow‐up of 25.2 months (IQR, 9.0‐51.6), and the 1‐year patient survival rate was 79.4%. LT can be considered as a feasible life‐saving option for the management of liver failure secondary to LCH in well‐selected patients.