IgG4-related sclerosing cholangitis associated with essential thrombocythemia:A case report  

作　　者：Zhi-Nian Wu Ru JI Ying Xiao Ya-Dong Wang Cai-Yan Zhao 

机构地区：[1]Department of Infectious Diseases,The Hebei Medical University Third Hospital,Shijiazhuang 050051,Hebei Province,China

出　　处：《World Journal of Clinical Cases》2024年第24期5589-5595,共7页世界临床病例杂志

基　　金：Natural Science Foundation of Hebei Province,No.H2023206042。

摘　　要：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.

关 键 词：Immunoglobulin G4-related sclerosing cholangitis Essential thrombocythemia Autoimmune pancreatitis Janus kinase 2 mutation GLUCOCORTICOIDS Case report 

分 类 号：R575.7[医药卫生—消化系统] R593.2[医药卫生—内科学]