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Rosai-Dorfman病7例并文献复习

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目的 探讨Rosai-Dorfman病的临床病理学特征、诊断及鉴别诊断,并复习相关文献.方法 回顾性研究攀枝花市中心医院2014-2023年诊断为Rosai-Dorfman病的病例,所有病例均由3位高年资病理审核医师复阅.结果7例患者,年龄7~54岁,男性3例,女性4例,男女比例3∶4.发生部位:鼻腔、臀部、右上肢上臂外侧、左侧顶部颅内、颈6椎管内、右乳各1例,左大腿、下腹部均发生1例.临床表现因发生部位不同而表现不一.镜下形态大致相同,可见显著增生的组织细胞,周边见大量淋巴细胞、浆细胞浸润,成片的组织细胞与成片的淋巴浆细胞呈交替排列的暗区和亮区分布,高倍镜下可见"伸入现象",即组织细胞内含有大量完整的淋巴细胞.免疫组化染色显示S-100、CD68、Cyclin-D1阳性,Langerin、AE1/AE3阴性.结论 Rosai-Dorfman病发病率低,发病年龄广,女性患者较多见,临床及影像学表现不典型,诊断主要依靠显微镜下特征及免疫组化染色结果.
Rosai-Dorfman disease:report of seven cases and review of literature
Objective To study the clinicopathological features,diagnosis,and differential diagnosis of Rosai-Dorfman disease(RDD)with review of the relevant literature.Methods A retrospective study was conducted on 7 cases of Rosai-Dorfman disease diagnosed in Panzhihua Central Hospital from 2014 to 2023.All cases were reviewed by three senior pathologists.Results Seven patients were 7-54 years old,including 3 males and 4 females,with a male to female ratio of 3∶4.Among the 7 cases,sites of occurrence included nasal cavity,hip,lateral upper arm of right upper limb,intracranial(left parietal lobe),spinal canal,right breast for one case each,and one case involved both left thigh and lower abdomen.Clinical manifestations varied according to the location of occurrence.Under the microscope,the morphology was roughly the same.A large number of histiocytes,lymphocytes and plasma cells were noted,showing alternating dark areas and bright areas.The emperipolesis(intracytoplasmic lymphocytes)was observed at higher magnifeication.Immunohistochemical staining showed histiocytes were positive for S-100,CD68 and cyclin-D1,but negative for Langerin and AE1/AE3.Conclusion The incidence rate of Rosai-Dorfman disease is low,the age of onset is various,the female patients are more common,the clinical and imaging presentations are not typical,and the diagosis mainly depends on microscopic features and immunohistochemistry.

Rosai-Dorfman diseaseClinicopathological featuresDiagnosis and differential diagnosis

肖华静、徐嘉忆、沈国菊

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攀枝花市中心医院病理科,四川攀枝花 617000

Rosai-Dorfman病 病理特征 诊断 鉴别诊断

2024

诊断病理学杂志
北京军区总医院

诊断病理学杂志

CSTPCD
影响因子:0.663
ISSN:1007-8096
年,卷(期):2024.31(1)
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