Wu ZN, JI R, Xiao Y, Wang YD, Zhao CY. IgG4-related sclerosing cholangitis associated with essential thrombocythemia: A case report. World J Clin Cases 2024; 12(24): 5589-5595 [DOI: 10.12998/wjcc.v12.i24.5589]
Corresponding Author of This Article
Ya-Dong Wang, PhD, Chief Doctor, Department of Infectious Diseases, The Hebei Medical University Third Hospital, No. 139 Ziqiang Road, Shijiazhuang 050051, Hebei Province, China. wangyadong@hebmu.edu.cn
Research Domain of This Article
Gastroenterology & Hepatology
Article-Type of This Article
Case Report
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/
World J Clin Cases. Aug 26, 2024; 12(24): 5589-5595 Published online Aug 26, 2024. doi: 10.12998/wjcc.v12.i24.5589
IgG4-related sclerosing cholangitis associated with essential thrombocythemia: A case report
Zhi-Nian Wu, Ru JI, Ying Xiao, Ya-Dong Wang, Cai-Yan Zhao
Zhi-Nian Wu, Ru JI, Ying Xiao, Ya-Dong Wang, Cai-Yan Zhao, Department of Infectious Diseases, The Hebei Medical University Third Hospital, Shijiazhuang 050051, Hebei Province, China
Author contributions: Wu ZN and Xiao Y accessed the literature and wrote the case; Ji R and Zhao CY provided material support for the study; Wang YD served as the corresponding author and critically reviewed and revised the manuscript; All authors wrote the manuscript and approved this version to be submitted.
Supported byNatural Science Foundation of Hebei Province, No. H2023206042.
Informed consent statement: The patient and his family provided their written informed consent and agreed to the publication of this case report.
Conflict-of-interest statement: The authors declare that there is no conflict of interest.
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: Ya-Dong Wang, PhD, Chief Doctor, Department of Infectious Diseases, The Hebei Medical University Third Hospital, No. 139 Ziqiang Road, Shijiazhuang 050051, Hebei Province, China. wangyadong@hebmu.edu.cn
Received: January 21, 2024 Revised: May 5, 2024 Accepted: June 4, 2024 Published online: August 26, 2024 Processing time: 172 Days and 0.3 Hours
Abstract
BACKGROUND
The complexity of immunoglobulin G4 (IgG4)-related diseases and their potential connection to hematologic malignancies remains unclear. This article provided a review of the diagnosis and treatment of a patient with IgG4-related sclerosing cholangitis (SC) and essential thrombocythemia (ET), along with an analysis of relevant literature to enhance comprehension of this disease.
CASE SUMMARY
A 56-year-old male was admitted to two hospitals with deteriorating jaundice and pruritus prior to hospitalization. Beyond our expectations, the patient was first diagnosed with IgG4-SC and ET with the Janus kinase 2 V617F mutation. Interestingly, the administration of acetate prednisone significantly resulted in improvements in both IgG4-SC and ET. Clinicians need to pay attention to immune disorders and inflammation as they contribute to the development of various disease phenotypes.
CONCLUSION
When IgG4-SC is suspected without histopathological evidence, diagnostic therapy and long-term regular follow-up can lead to positive treatment outcomes. Clinicians should be mindful of the potential presence of concurrent hematologic diseases in patients with immune disorders.
Core Tip: The case diagnosed with immunoglobulin G4 (IgG4)-related sclerosing cholangitis (SC) and essential thrombocythemia was first reported. In this article, we described the clinical features of this case and reported the diagnosis and treatment process and prognosis. The relationship between IgG4-SC and Janus kinase 2 V617F mutation diseases was analyzed and summarized by retrieving literature.
