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IgG4相关疾病误诊为多中心型Castleman病3例并文献回顾

焦洋 张文 黄晓明 曾学军

中华临床免疫和变态反应杂志2013,Vol.7Issue(2):154-159,6.
中华临床免疫和变态反应杂志2013,Vol.7Issue(2):154-159,6.

IgG4相关疾病误诊为多中心型Castleman病3例并文献回顾

IgG4-related Disease Misdiagnosed as Multicentric Castleman's Disease: A Three-cases Report and Literature Review

焦洋 1张文 2黄晓明 1曾学军1

作者信息

  • 1. 中国医学科学院,北京协和医学院,北京协和医院普通内科,北京,100032
  • 2. 中国医学科学院,北京协和医学院,北京协和医院风湿免疫科,北京,100032
  • 折叠

摘要

Abstract

Objective To analyze the clinical features of IgG4-related disease and determine the reasons why The patients with IgG4-related disease are misdiagnosed with multicentric Castleman's disease. Methods We retrospectively reviewed the medical records of the three patients with IgG4-related disease, who were admitted to Peking Union Medical College Hospital ( PUMCH) and had been misdiagnosed as multicentric Castleman' s disease. Results All three patients were males aged 53 ~ 57. They presented with generalized lymphadenopathy and multiple organ involvement. Biopsy results of their lymph nodes were consistent with multicentric Castleman' s disease. However, all their serum IgG4 levels were significantly elevated. Fluorodeoxyglucose (FDG) positron emission tomography-computed tomography (PET-CT) scan detected abnormal FDG uptake in multiple organs in all patients; Immunohistochemical analysis of biopsy specimen from two patients revealed that the IgG4-positive/IgG-positive plasma cell ratios were more than 50% . They were diagnosed with IgG4-related disease. High-dose corticosteroid with cyclophosphamide resulted in marked resolution of the physical, serological, and imaging abnormalities in all patients. Conclusions IgG4-related disease sometimes can be misdiagnosed as multicentric Castleman' s disease, if only based on clinical features and conventional pathological examination. It is necessary to strengthen the recognition of the disease. Measurement of serum IgG4 levels, PET-CT scan and specific immunohistochemical analysis for determining the IgG4-positive/IgG-positive plasma cell ratio are helpful in the diagnosis of IgG4-related disease.

关键词

IgG4相关疾病/Castleman病/免疫组化分析

Key words

IgG4-related disease/ Castleman' s disease/ immunohistochemical analysis

分类

医药卫生

引用本文复制引用

焦洋,张文,黄晓明,曾学军..IgG4相关疾病误诊为多中心型Castleman病3例并文献回顾[J].中华临床免疫和变态反应杂志,2013,7(2):154-159,6.

基金项目

中华医学会临床医学研究专项基金(12040680368),北京市科技新星(2008B49) (12040680368)

中华临床免疫和变态反应杂志

OACSTPCD

1673-8705

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