IgG4相关性疾病胆管受累并发血小板减少1例

IgG4-Related Disease Involving the Bile Ducts Complicated by Thrombocytopenia: A Case Report

摘要: 目的：报道1例IgG4相关性疾病(IgG4-related disease, IgG4-RD)以胆管肿物为主要表现，并发血小板减少的患者，探讨该病的临床特征及诊疗。病历摘要：74岁男性患者，因“皮肤、巩膜黄染伴小便发黄4月余”住院，根据症状、辅助检查、胆管肿物活检考虑为IgG4-RD。入院后查血发现血小板减少，完善骨髓穿刺后排除原发性血液系统疾病，给予激素、免疫抑制剂、免疫球蛋白及输血小板治疗。后复查患者IgG4较前下降、血小板计数上升，予患者口服甲泼尼龙60 mg/d，环孢素150 mg/d，羟氯喹400 mg/d，利可君片60 mg/d维持治疗出院。患者出院后规律于门诊复诊、调整用药，随访3月，患者IgG4指标正常，血小板计数稳定。结论：IgG4-RD患者可出现多系统受累，与肿瘤鉴别困难且易与肿瘤并发，本例患者诊治过程提示对于不典型病例应注意结合影像学及病理活检甄别病情，及早启动原发病治疗。

Abstract: Objective: To report a case of IgG4-related disease (IgG4-RD) presenting primarily as a biliary duct mass complicated by thrombocytopenia, and to discuss the clinical characteristics, diagnosis, and management of this condition. Case Summary: A 74-year-old male was hospitalized due to “jaundice of the skin and sclera accompanied by dark urine for over 4 months”. Based on clinical manifestations, auxiliary examinations, and biliary duct biopsy, the patient was diagnosed with IgG4-RD. During hospitalization, laboratory tests revealed thrombocytopenia. A bone marrow aspiration was performed, which ruled out primary hematological disorders. Treatment included glucocorticoids, immunosuppressants, intravenous immunoglobulin, and platelet transfusions. Follow-up tests showed a decrease in serum IgG4 levels and an increase in platelet count. The patient was discharged on a maintenance regimen of oral methylprednisolone 60 mg/day, cyclosporine 150 mg/day, hydroxychloroquine 400 mg/day, and leucogen 60 mg/day. He attended regular outpatient follow-ups for medication adjustments. After three months of follow-up, his IgG4 levels normalized and platelet count remained stable. Conclusion: IgG4-RD can involve multiple organ systems and is often difficult to distinguish from malignancy, with which it may also coexist. The diagnostic and therapeutic process in this case highlights the importance of integrating imaging and pathological biopsy for atypical presentations to initiate prompt treatment of the underlying disease.