结外Rosai-Dorfman病3例临床病理分析

目的探讨原发性淋巴结外Rosai-Dorfman病的临床病理学特征、诊断及鉴别诊断。方法分析3例结外Rosai-Dorfman病的临床特点,并行HE和免疫组化染色观察。结果3例中男性2例,女性1例,年龄13~63岁,均为多发性,病变位于中枢神经系统、涎腺及皮肤。光镜下增生的组织细胞与浸润的淋巴细胞、浆细胞形成浅、深相间的结构特点,特征性的组织细胞体积巨大、胞质丰富,内见形态完整的淋巴细胞、浆细胞及中性粒细胞等,组织细胞表达S-100、CD68和Mac387。结论结外Rosai-Dorfman病是一种少见的组织细胞增生性病变,有特定的病理学特征,系统累及可能性较小,预后较好。组织学上应与Langerhans细胞组织细胞增生症、慢性炎症等病变鉴别。

Extranodal Rosai-Dorfman disease:a clinicopathological analysis of 3 cases

Objective To study the clinicopathological features,diagnosis and differential diagnosis of extranodal Rosai-Dorfman disease(RDD). Methods Clinical data,sections of hematoxylin-eosin and immunohistochemical staining were analyzed in 3 cases of RDD. Results The patients were 2 males and 1 female,aged from 13 to 63 years.The lesions were located in the central nervous system,salivary glands and skin,rspectively.Histologically,the lesions were characterized by variable numbers of large histiocytes with emperipolesis,accompanied by infiltration of lymphocytes and plasma cells.The histiocytes were positive for S-100 protein,CD68 and Mac387. Conclusion Extranodal RDD is a very rare histiocytic proliferative disorder which has distinct morphologic feature.Systemic involvement is rare and its prognosis good.It should be differentiated from other types of histiocytosis such as langerhans cell histiocytosis,chronic inflammation and other lesions.