Wu Zhi-Nian, Ji Ru, Xiao Ying, Wang Ya-Dong, Zhao Cai-Yan
Department of Infectious Diseases, The Hebei Medical University Third Hospital, Shijiazhuang 050051, Hebei Province, China.
World J Clin Cases. 2024 Aug 26;12(24):5589-5595. doi: 10.12998/wjcc.v12.i24.5589.
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.
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.
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.
免疫球蛋白G4（IgG4）相关疾病的复杂性及其与血液系统恶性肿瘤的潜在联系仍不明确。本文对一名患有IgG4相关硬化性胆管炎（SC）和原发性血小板增多症（ET）的患者的诊断和治疗进行了综述，并对相关文献进行分析，以增进对该疾病的理解。
一名56岁男性在入院前因黄疸和瘙痒加重入住两家医院。出乎我们意料的是，该患者最初被诊断为IgG4-SC和伴有Janus激酶2 V617F突变的ET。有趣的是，给予醋酸泼尼松显著改善了IgG4-SC和ET。临床医生需要关注免疫紊乱和炎症，因为它们会导致各种疾病表型的发展。
当怀疑IgG4-SC但无组织病理学证据时，诊断性治疗和长期定期随访可带来积极的治疗结果。临床医生应注意免疫紊乱患者中潜在并发血液系统疾病的存在。
