Department of Respiratory Medicine, University of Occupational and Environmental Health, Kitakyushu, Japan.
Tohoku J Exp Med. 2012 Jul;227(3):231-235. doi: 10.1620/tjem.227.231.
Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a rare non-neoplastic disease that is characterized by a proliferation of histiocytes mostly in lymph nodes. However, the etiological mechanism of RDD still remains unclear. Intrathoracic manifestations of RDD are only observed in 2% of patients with RDD. Spontaneous remission was reported in about 20% of patients with RDD; however, there are no reports of an intrathoracic manifestation of RDD that showed a spontaneous remission within a short period of time. A 64-year-old Japanese female with dry cough and left chest pain was introduced to our hospital, and computed tomography revealed a pulmonary nodular lesion and enlarged mediastinal lymph nodes. The bronchial specimen obtained from the abnormal mucosal lesion showed massive infiltration of histiocytes underneath the bronchial epithelium and emperipolesis, a typical pathological finding in RDD, which is characterized by the presence of histiocyte-like cells engulfing intact lymphocytes. These histiocytes were positive for S-100 (one of the known positive markers of RDD) and for CD68 (a marker for various cells of the macrophage lineage). All these findings are consistent with the diagnosis of RDD. These radiological and endoscopic findings spontaneously resolved within four months without any treatment. In conclusion, clinicians should be aware of this disease as one of differential diagnoses of pulmonary nodules in combination with mediastinal lymph node enlargements, especially in order to differentiate it from primary lung cancer.
罗道尔夫-多夫曼病（RDD），又称窦组织细胞增生伴巨大淋巴结病，是一种罕见的非肿瘤性疾病，其特征是组织细胞主要在淋巴结中增殖。然而，RDD 的病因机制仍不清楚。RDD 的胸腔内表现仅见于 2%的 RDD 患者。约 20%的 RDD 患者自发缓解；然而，尚无 RDD 胸腔内表现短期内自发缓解的报道。一位 64 岁的日本女性因干咳和左侧胸痛被介绍到我院，计算机断层扫描显示肺部结节状病变和纵隔淋巴结肿大。从异常黏膜病变中获得的支气管标本显示，支气管上皮下有大量组织细胞浸润和吞噬现象，这是 RDD 的典型病理表现，其特征是组织细胞样细胞吞噬完整的淋巴细胞。这些组织细胞 S-100（RDD 的已知阳性标志物之一）和 CD68（巨噬细胞谱系各种细胞的标志物）阳性。所有这些发现都符合 RDD 的诊断。这些影像学和内镜检查结果在没有任何治疗的情况下在四个月内自发消退。总之，临床医生应将其视为肺部结节合并纵隔淋巴结肿大的鉴别诊断之一，特别是为了将其与原发性肺癌区分开来。
