一种新认识的IgG4相关性淋巴结病组织学类型 形态学谱系的拓展

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  ——本文经《美国外科病理学杂志》授权发布,其他媒体转载或引用须经《美国外科病理学杂志》同意,否则追究法律责任。

  摘要:免疫球蛋白(immunoglobulin,Ig)G4相关性的硬化性疾病是一类纤维炎性病变,其特点是在多个解剖学部位形成瘤样病变和血清IgG4水平的升高。IgG4相关性淋巴结病定义为lgG4相关性硬化性疾病所致的淋巴结病变,该疾病可表现为5种组织学模式:(1)多中心Castleman病样;(2)反应性淋巴滤泡增生;(3)淋巴滤泡间伴免疫母细胞的浆细胞增多症;(4)进行性生发中心样转化和(5)炎性假瘤样。本研究中,我们报道了1例发生在37岁男性患者的IgG4相关性淋巴结病新的组织学形态,我们将其定义为“传染性单核细胞增多症样”。这种组织学形态的特点是大量成熟的浆细胞、浆细胞样细胞、大的嗜碱性转化淋巴细胞(免疫母细胞)、小-中等大小的淋巴细胞和组织细胞浸润破坏淋巴结结构。局灶可见血管周纤维化和核碎裂伴纤维蛋白原沉积。EB病毒(Epstein-Barr virus,EBV)编码的小RNA原位杂交显示散在1%~2%阳性小淋巴细胞。在激素治疗1个月后,血清中IeG4的初始值(>4400mg/dL)下降了50%。随访1年,患者病情稳定。该病例报道拓展了IgG4相关性淋巴结病的形态学谱系。
  关键词:IgG4相关性硬化性疾病,IgG4相关性淋巴结病,传染性单核细胞增多症样,新的形态学谱系
  AmJ Surg Pathol 2018;42:977-982
  美国外科病理学杂志中文版2019年第1期全文No.4
  唐立华 翻译 段亚琦 审校





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