模仿IgG4相关淋巴结病的多中心型Castleman病：易误诊病例的鉴别诊断及文献复习

来源期刊：广州医药 | 121-126 发布时间：2024-03-06 收稿时间：2025/11/13 18:50:39 阅读量：26

作者：: 李春艳,

刘国荣,

常丽君,

李秀博,

丁文双,

关键词：: Castleman病 IgG4相关淋巴结病淋巴结浆细胞鉴别诊断; Castleman disease IgG4-related lymphadenopathy lymph node plasma cells differential diagnosis

DOI：: 10.3969/j.issn.1000-8535.2024.02.003

收稿时间：: 2023-03-09
修订日期：
接收日期：
引用总数：: 1

目的通过学习1例少见的组织学特征与IgG4相关性淋巴结病类似的浆细胞型特发性多中心型Castleman病（PC-iMCD）,总结两种易误诊疾病的鉴别要点,提高病理诊断水平。方法回顾性分析1例PC-iMCD患者临床资料,常规苏木素-伊红（HE）染色分析淋巴结组织结构及细胞形态,免疫组织化学染色及原位杂交分析免疫表型及EB病毒（EBV）感染状态,并结合文献分析讨论其与IgG4相关淋巴结病的鉴别诊断。结果 48岁女性患者,临床表现为口干、多饮、皮肤瘙痒伴全身多处淋巴结肿大。实验室检查血清IgG、IgA、IgM及IgE水平均升高,血清IgG4显著升高（14.7 g/L）,白介素- 6（IL-6）异常升高（150.84 pg/mL）。病理检查显示淋巴结生发中心萎缩,套区淋巴细胞呈“洋葱皮”样围绕生发中心排列,滤泡间区扩张,其内见大量成熟的浆细胞呈片状浸润,灶区见含铁血黄素沉积及血管增生;免疫组化染色显示IgG4阳性浆细胞数大于100/高倍视野,IgG4阳性细胞/IgG阳性细胞比值>40%,Kappa及Lambda轻链呈非限制性表达;EB病毒编码RNA原位杂交（EBER）阴性。结论部分PC-iMCD与IgG4相关淋巴结病具有相似的组织病理学特征,单纯根据组织学及免疫表型难以将两者鉴别,正确诊断需结合IgG4相关病变诊断标准、排除性诊断标准、临床表现及实验室检查综合判断。

Objective By studying a rare case of the plasma cell type idiopathic multicentric Castleman disease（PC-iMCD）with histological characteristics similar to IgG4-related lymphadenopathy,the differential points of the two easily misdiagnosed diseases were summarized to improve the level of pathological diagnosis．Methods The clinical data of one patient with PC-iMCD were collected,the structure alteration and cell morphology were observed by hematoxylin-eosin（HE）stains．The immunophenotype of cells was marked by immunohistochemical staining and the infections status of EB virus was detected by in situ hybridization．Besides,the differential diagnosis between IgG4-RD and PC-iMCD were analyzed and discussed based on literature analysis．Results This article reported a 48-year-old female who was admitted to the hospital with dry mouth,polydipsia,skin itching and multiple lymphadenopathy．The levels of various classes of serum immunoglobulin were all increased,such as IgG,IgA,IgM and IgE．Specially,the serum IgG4 was also significantly increased（14.7 g/L）and interleukin 6（IL-6）was abnormally raised（150.84 pg/mL）．The pathological examination indicated that the lymph node germinal center was atrophied and mantle zones were expanded which were composed of concentric rings of lymphocytes in an “onion skinning” appearance．Besides,the interfollicular area was expanded in which mature plasma cells were infiltrated in sheet-like,hemosiderin was deposited as well as the vessels were proliferated．Immunohistochemistry showed that the number of IgG4-positive plasma cells was >100/HPF,IgG4/IgG-positive cells ratio was >40%,and Kappa and Lambda light chains were expressed unrestrictedly．In situ hybridization revealed that the expression of EBER was negative．Conclusions PC-iMCD and IgG4-related lymphadenopathy shares similar histopathological characteristics and it’s challenging to distinguish these two diseases by their histology and immunophenotype．In conclusion,the correct diagnosis should be combined with the diagnostic criteria,exclusion diagnostic criteria,clinical manifestations and related laboratory examinations．

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