Case Report
Copyright ©The Author(s) 2025. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Hepatol. Jul 27, 2025; 17(7): 106253
Published online Jul 27, 2025. doi: 10.4254/wjh.v17.i7.106253
Autoimmune hepatitis with syncytial giant cells in chronic lymphocytic leukemia: A case report and literature review
Marta Giacomelli, Simone Carotti, Federico Vozella, Federica Pagliei, Chiara Taffon, Andrea Baiocchini, Francesco Luigi Gambaro, Antonio Picardi, Umberto Vespasiani-Gentilucci, Giovanni Galati
Marta Giacomelli, Department of Internal Medicine, Department of Medicine and Surgery, Università Campus Bio-Medico di Roma, Rome 00128, Lazio, Italy
Simone Carotti, Microscopic and Ultrastructural Anatomy Research Unit, Department of Medicine and Surgery, Università Campus Bio-Medico di Roma, Rome 00128, Lazio, Italy
Simone Carotti, Department of Predictive Molecular Diagnostics, Fondazione Policlinico Universitario Campus Bio-Medico, Rome 00128, Lazio, Italy
Federico Vozella, Operative Research Unit of Hematology, Fondazione Policlinico Universitario Campus Bio-Medico, Rome 00128, Lazio, Italy
Federica Pagliei, Research Unit of Hematology, Department of Medicine and Surgery, Università Campus Bio-Medico di Roma, Rome 00128, Lazio, Italy
Chiara Taffon, Francesco Luigi Gambaro, Research Unit of Anatomical Pathology, Department of Medicine and Surgery, Università Campus Bio-Medico Di Roma, Rome 00128, Lazio, Italy
Chiara Taffon, Francesco Luigi Gambaro, Anatomical Pathology Operative Research Unit, Fondazione Policlinico Universitario Campus Bio-Medico, Rome 00128, Lazio, Italy
Andrea Baiocchini, UOC Anatomia Patologica Ospedale, San Camillo Forlanini, Circ.ne Gianicolense, Rome 00128, Lazio, Italy
Antonio Picardi, Umberto Vespasiani-Gentilucci, Giovanni Galati, Operative Research Unit of Clinical Medicine and Hepatology, Fondazione Policlinico Universitario Campus Bio-Medico, Rome 00128, Italy
Antonio Picardi, Umberto Vespasiani-Gentilucci, Research Unit of Internal Medicine, Department of Medicine and Surgery, Università Campus Bio-Medico Di Roma, Rome 00128, Lazio, Italy
Author contributions: Giacomelli M drafted the manuscript; Galati G and Vozella F were responsible for the clinical follow-up and collected the data of the patient presented in this case report; Carotti S contributed to conceptualization and supervision; All authors read and approved the final version of the manuscript to be published.
Informed consent statement: Informed written consent was obtained from the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: The authors have no conflicts of interest to declare.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
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: Giovanni Galati, Consultant, Operative Research Unit of Clinical Medicine and Hepatology, Fondazione Policlinico Universitario Campus Bio-Medico, Via Alvaro del Portillo 200, Rome 00128, Italy. g.galati@policlinicocampus.it
Received: February 20, 2025
Revised: March 26, 2025
Accepted: June 24, 2025
Published online: July 27, 2025
Processing time: 155 Days and 12.5 Hours
Abstract
BACKGROUND

Hepatic manifestations in chronic lymphocytic leukemia (CLL) are common: Elevation of liver enzymes frequently occurs, and differential diagnosis is often challenging. Liver infiltration by leukemic cells, primary and secondary hepatic malignancies, drug-induced hepatotoxicity, immunological disorders, and infections have been reported. Nevertheless, syncytial giant cell hepatitis (GCH) as a manifestation of autoimmune hepatitis in patients with CLL is an extremely rare condition, currently reported only in anecdotal cases.

CASE SUMMARY

Here, we report the case of a 62-year-old Caucasian woman affected by CLL, who developed GCH with peculiar histopathological features. The patient was evaluated for abnormal liver test results. Liver histology revealed significant inflammatory lymphomononuclear infiltrates with a plasma cell component, widespread syncytial changes in the hepatocytes with gigantocellular features, hepatocyte rosettes, and the typical feature of emperipolesis, consistent with a diagnosis of GCH. The patient was treated with corticosteroids and mycophenolate mofetil, resulting in a complete biochemical response.

CONCLUSION

Early histological diagnosis of GCH is crucial in patients with CLL, with mycophenolate mofetil representing a promising treatment option.

Keywords: Chronic lymphocytic leukemia; Autoimmune hepatitis; Syncytial giant cell hepatitis; Toll-like receptor 4; Multinucleated liver cells; Mycophenolate mofetil; Liver biopsy; Case report

Core Tip: The association between chronic lymphocytic leukemia (CLL) and syncytial giant cell hepatitis (GCH) is rare, with only a few cases reported in the literature. For this reason, early diagnosis is essential to prevent liver fibrosis and distinguish GCH from other causes of liver disorders. Active immunosuppressive therapy may be beneficial for patients with GCH, with mycophenolate mofetil emerging as a promising therapeutic option. Several studies have suggested a modified expression of toll-like receptors (TLRs) in CLL, potentially contributing to autoimmune complications. Further studies are needed to evaluate the role of TLR4 in autoimmune hepatitis development in patients with CLL.