罗莎伊-多夫曼病模仿IgG4相关疾病
Okan Cetin1, Sertac Yetisir2, Timur Selcuk Akpinar2
1Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University, 34093, Istanbul, Turkey. okancetin@istanbul.edu.tr.
Wiener medizinische Wochenschrift (1946)
|September 9, 2025
概括
罗莎-多夫曼病 (RDD) 可以模仿IgG4相关的疾病 (IgG4-RD),带来诊断挑战. 切割活检和临床评估对于准确区分RDD和IgG4-RD至关重要.
科学领域:
- 囊细胞性疾病 囊细胞性疾病
- 免疫病理学 免疫病理学
背景情况:
- 罗莎-多尔夫曼病 (RDD) 是一种罕见的囊细胞性疾病.
- RDD可以呈现与IgG4相关疾病 (IgG4-RD) 重叠的特征,包括淋巴腺病变和升高的IgG4水平.
- 区分RDD与IgG4-RD对于适当的患者管理具有临床意义.
研究的目的:
- 要突出诊断的挑战,区分罗莎伊-多夫曼病与IgG4相关的疾病.
- 强调在RDD诊断中特定的组织病理学发现和活检技术的重要性.
主要方法:
- 一个69岁的妇女的病例报告,患有泛性淋巴腺病和宪法症状.
- 最初的诊断怀疑IgG4-RD基于核心针活检.
- 通过切除性淋巴结活检确认RDD的诊断,揭示了emperipolesis和S100阳性囊细胞.
主要成果:
- 核心针活检发现最初暗示IgG4-RD.
- 切除淋巴结活检显示了RDD的病理特征,包括emperipolesis.
- 发现IgG4水平升高,进一步使初始诊断评估复杂化.
结论:
- 在RDD和IgG4-RD之间存在显著的临床和病理学重叠.
- 切除活检优于核心针活检,用于在模糊的情况下诊断RDD.
- 准确区分RDD和IgG4-RD需要结合详细的临床评估和最终的组织病理学分析.
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