Rosai-Dorfman病的临床病理、免疫表型和病原学观察  

Rosai-Dorfman disease:clinicopathologic,immunohistochemical and etiologic study of 16 cases

作　　者：于建渤[1] 刘卫平[1] 左卓[1] 唐源[1] 廖殿英[1] 纪洪[1] 白燕琼[2] 李世辉[2] 鲁常青[3] 罗红波[4] 

机构地区：[1]四川大学华西医院病理科,成都610041 [2]四川省简阳市人民医院病理科 [3]江苏省常州市第一人民医院病理科 [4]四川省安岳县人民医院病理科

出　　处：《中华病理学杂志》2007年第1期33-38,共6页Chinese Journal of Pathology

摘　　要：目的观察 Rosai-Dorfman 病的临床病理特征,探讨多种抗原标记的免疫表型及HHV8-DNA、HPV-DNA 和 EBER 的表达及其与病因的关系,并比较国内外 Rosai-Dorfman 病的发病特征。方法对16例 Rosai-Dorfman 病进行临床病理回顾性研究;9例行免疫表型检测,包括 CD163、CD68(PG-M1)、CD21、CD1a、CD20、CD45RO、CD8、CD4、S100、M-CSF 和 HHV8;用原位杂交技术进行EBV-mRNA 和 HPV-DNA 检测。结果 (1)男女之比为4.33:1。只有淋巴结病变的占62.5%(10/16),颈淋巴结多见,50%的病例有多部位淋巴结累及;结外病变占37.5%(6/16)。结外病变者的局部复发率较高。(2)淋巴结病变以明显扩张的淋巴窦内有不等量体积大、多角形、胞质内含有淋巴细胞和浆细胞的组织细胞为特征;结外病变均有不同程度纤维组织增生和以浆细胞为主的混合炎细胞浸润,胞质内含有淋巴细胞和浆细胞的大多角形组织细胞数量多寡不一,呈簇状或散在分布,且随病程进展,纤维组织的量渐多,特征性组织细胞数量渐少。(3)病变组织中大多角形组织细胞均表达 S-100蛋白,以及 CD68PG-M1、CD163和 M-CSF,均不表达 CD1a 和 CD21;组织细胞吞人的有 T 和 B细胞,以 T 细胞为多,且 CD4和 CD8表型细胞均有。9例均为 HHV8和 EBEV 阴性。(4)国内报道共62例,其中仅淋巴结病变34例,仅结外病变18例,淋巴结和结外同时存在病变10例。比较国内外文献,国外患者年龄较国内的年轻10岁以上,且女性比例较高。结论 Rosai-Dorfman 病在国内较少。结外 Rosai-Dorfman 病的诊断有一定难度。HHV8-DNA、HPV-DNA 和 EBER 的表达与病因关联不明确。国内外患者的发病年龄和性别构成不同。Objectives To study the elinicopathologic features of Rosai-Dorfman disease （RDD） , expression of various antigens, human herpes virus type 8 （ HHV8 ） , human papillomavirus （HPV） -DNA and Epstein-Barr virus （EBV）-mRNA, and compare the findings with those in the literature. Methods The clinicopathologic findings of 16 Rosai-Dorfman disease cases were retrospectively reviewed. Immunohistochemical study for S-100 protein, CD68 （PG-M1）, CD163, CD21, CD1a, CD20, CD45RO, CD4, CD8, M-CSF and HHV8 was carried out in 9 of the 16 cases. In-situ hybridization for EBV-mRNA and HPV-DNA was also performed. Results The male-to-female ratio of the patients was 4.33 ： 1. Amongst the 16 cases studied, 62, 5% （10/16） presented nodal RDD, with cervical lymph node predominantly involved. Half of these cases had affected lymph nodes in more than one anatomic site. Extranodal RDD represented 37, 5% （6/16） of the cases. The relapse rate of extranodal RDD was higher than that of nodal RDD, Histologically, nodal RDD was characterized by dilated sinuses filled with large polygonal histiocytes which contained lymphocytes and plasma cells. For extranodal lesions, various degrees of stromal fibrosis were seen in association with mixed inflammatory cells （ especially plasma cells ）. The large polygonal hisfiocytes varied in number and were distributed in clusters or patches. Immunohistochemical study showed that the abnormal histiocytes were strongly positive for S-100 protein. They also expressed CD68, CD163 and M-CSF, but were negative for CDla, CD21 and HHV8. The lymphocytes in cytoplasm of these histiocytes were positive for both T and B cell markers （with T cell predominance, including a mixture of CD4- and CD8-positive cells）. HPV-DNA and EBV-mRNA were not detected by in-situ hybridization. To date, 62 cases of RDD have been reported in China's Mainland, including 34 cases of nodal RDD and 18 cases of extranodal RDD. The remaining 10 cases involved both lymph nodes and extranodal sites. Compared wit

关 键 词：组织细胞增多症 窦 免疫表型分型 DNA探针 HPV 

分 类 号：R55[医药卫生—血液循环系统疾病]