Hsiao Cheng-Hsiang, Tsai Tsen-Fang, Yang Ting-Hua, Liu Chia-Ming
Department of Pathology, National Taiwan University Hospital, and National Taipei College of Nursing, Taipei, Taiwan.
J Formos Med Assoc. 2006 Sep;105(9):701-7. doi: 10.1016/S0929-6646(09)60197-2.
BACKGROUND/PURPOSE: Rosai-Dorfman disease (RDD) is a rare histiocytic proliferative disorder that usually presents as cervical lymphadenopathy. Extranodal involvement occurs in up to 40% of patients. The disease is most prevalent in blacks and rare in Asians. This study analyzed the characteristics of RDD in patients from Taiwan.
Fourteen patients with a diagnosis of RDD were identified by review of records from 1995 to 2004 at National Taiwan University Hospital. Tissue sections from each patient were reviewed and immunohistochemical staining was performed. Data on clinical presentations, associated diseases, treatment and outcome were analyzed. In situ hybridization for Epstein-Barr virus (EBV)-encoded small RNAs (EBER-1) was also performed. Sets of primers specific for the conservative region of bacterial 16S-rDNA, IS6110 of Mycobacterium tuberculosis complex and consensus region of human herpes virus (HHV) DNA polymerase genome were used to detect the presence of these infectious agents in the specimens.
There were six men and eight women with a mean age of onset of 44 years. Nine patients presented with skin lesions, four with lymph node involvement and one with nasal tumor. All lesions followed a chronic and indolent course. Most of the lesions regressed spontaneously, and no patients died as a result of this disease during follow-up. Three patients had associated immune-mediated disease, i.e. hemolytic anemia, ankylosing spondylitis and asthma. Two patients had a history of tuberculosis. Histologically, all lesions were characterized by a mixed infiltrate of large pale histiocytes, abundant plasma cells and lymphocytes regardless of the site of involvement. The strong immunoreactivities of these histiocytes to S-100 protein, CD68 and CD14 with occasional lymphophagocytosis were helpful in confirming the diagnosis, polymerase chain reaction analysis of 16S-rDNA, IS6110 and HHV gene and in situ hybridization for EBV were all negative.
RDD in Taiwan is characterized by older age of onset compared to Western countries (44 years vs. 20 years) and more frequent extranodal involvement. The skin was the most common site of extranodal involvement, with about two-thirds of patients presenting with cutaneous lesions. There was no evidence of bacterial, mycobacterial or HHV infection in this series.
背景/目的：罗萨伊-多夫曼病（RDD）是一种罕见的组织细胞增生性疾病，通常表现为颈部淋巴结病。结外受累见于高达40%的患者。该疾病在黑人中最为常见，在亚洲人中罕见。本研究分析了台湾患者RDD的特征。
通过回顾1995年至2004年台湾大学附属医院的记录，确定了14例诊断为RDD的患者。对每位患者的组织切片进行复查并进行免疫组化染色。分析临床表现、相关疾病、治疗及预后的数据。还进行了爱泼斯坦-巴尔病毒（EBV）编码的小RNA（EBER-1）的原位杂交。使用针对细菌16S-rDNA保守区、结核分枝杆菌复合群的IS6110以及人类疱疹病毒（HHV）DNA聚合酶基因组共有区的引物组来检测标本中这些感染因子的存在。
有6名男性和8名女性，平均发病年龄为44岁。9例患者出现皮肤病变，4例有淋巴结受累，1例有鼻部肿瘤。所有病变均呈慢性、惰性病程。大多数病变自发消退，随访期间无患者因该病死亡。3例患者有相关的免疫介导疾病，即溶血性贫血、强直性脊柱炎和哮喘。2例患者有结核病史。组织学上，无论受累部位如何，所有病变均以大量淡染组织细胞、丰富浆细胞和淋巴细胞的混合浸润为特征。这些组织细胞对S-100蛋白、CD68和CD14的强免疫反应性以及偶尔的噬淋巴细胞现象有助于确诊，16S-rDNA、IS6110和HHV基因的聚合酶链反应分析以及EBV的原位杂交均为阴性。
与西方国家相比，台湾的RDD发病年龄较大（44岁对20岁），结外受累更频繁。皮肤是结外受累最常见的部位，约三分之二的患者有皮肤病变。本系列中没有细菌、分枝杆菌或HHV感染的证据。
